AIFM1

UniProt ID: O95831
Organism: Homo sapiens
Review Status: DRAFT
πŸ“ Provide Detailed Feedback

Gene Description

AIFM1 encodes apoptosis-inducing factor, a FAD-containing mitochondrial NADH oxidoreductase with additional roles in protein import and regulated cell death. The mature full-length protein is anchored in the inner mitochondrial membrane, with its major domain exposed to the intermembrane space. NADH-driven dimerization enables AIF to anchor and activate CHCHD4/MIA40, supporting oxidative folding and import through the mitochondrial disulfide relay. Following appropriate death signals, further proteolytic processing releases AIF for cytoplasmic and nuclear redistribution; DNA and poly(ADP-ribose) interactions contribute to chromatin-associated cell death. Alternative products separate mitochondrial redox activity from proapoptotic activity. AIFM1 variants cause a spectrum of X-linked mitochondrial, peripheral neurological and auditory disorders.

Existing Annotations Review

GO Term Evidence Action Reason
GO:0002931 response to ischemia
IEA
GO_REF:0000107
UNDECIDED
Summary: The rat-to-human ischemia assertion requires its original experiment and context.
Reason: The supplied donor is rat Aifm1 (UniProtKB:Q9JM53; Ensembl ENSRNOP00000008503), not a paralog. Human AIF has established redox, import and death-effector functions, but the exact donor experiment underlying this ischemia response was not recovered through the available annotation interfaces. General AIF activities do not establish the particular stimulus, tissue and response mechanism. Retain uncertainty rather than label the donor annotation wrong or infer human conservation solely from orthology.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
UniProtKB:Q9JM53 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for ischemia remain unresolved. No functional divergence or bad-source conclusion is established.
ensembl:ENSRNOP00000008503 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for ischemia remain unresolved. No functional divergence or bad-source conclusion is established.
GO:0003677 DNA binding
IDA
PMID:27178839
Structure/Function Relations in AIFM1 Variants Associated wi...
ACCEPT
Summary: AIF directly binds DNA through its proapoptotic domain.
Reason: The source studies human AIF variants and is the curated basis for sequence-independent DNA binding in UniProt O95831. Its local cache is abstract-only, so the original IDA is retained with curator deference and independent support from recombinant human WT/mutant DNA-retardation experiments in PMID:23217327 Figure 3E. DNA binding is distinguished from intrinsic nuclease activity and sequence-specific transcriptional regulation.
GO:0003954 NADH dehydrogenase activity
EXP
PMID:16644725
Identification and characterization of AIFsh2, a mitochondri...
ACCEPT
Summary: AIFsh2 retains NADH oxidoreductase activity.
Reason: PMID:16644725 characterizes the mitochondrial AIFsh2 oxidoreductase domain and NADH-dependent activity. This supports generic-acceptor dehydrogenase chemistry without selecting a physiological acceptor. The assayed splice product does not induce nuclear apoptosis; its evidence is not generalized to every alternative product.
Supporting Evidence:
PMID:16644725
AIFsh2 exhibits similar NADH oxidase activity to AIF and generates reactive oxygen species.
GO:0003954 NADH dehydrogenase activity
EXP
PMID:23217327
Cowchock syndrome is associated with a mutation in apoptosis...
ACCEPT
Summary: Recombinant human AIF catalyzes NADH-dependent acceptor reduction.
Reason: Full PMID:23217327 Table 1 compares WT and E493V human AIF reduction of DCIP, ferricyanide and cytochrome c. GO:0003954 specifies NADH-to-acceptor chemistry, not respiratory complex I or proton pumping. These assays support the activity while leaving the physiological electron acceptor unresolved.
Supporting Evidence:
PMID:23217327
The c.1478A>T (p.Glu493Val) mutation found in the family reported here alters the redox properties of the AIF protein and results in increased cell death via apoptosis
GO:0003954 NADH dehydrogenase activity
EXP
PMID:24914854
Structural insights into the coenzyme mediated monomer-dimer...
ACCEPT
Summary: Human AIF couples NADH reduction to a redox-dependent structural transition.
Reason: PMID:24914854 studies human AIF coenzyme binding, reduction and monomer-dimer equilibrium; the curated UniProt reaction retains NADH-to-acceptor reduction. Independent full human acceptor-reduction assays in PMID:23217327 establish the same chemistry. Neither source assigns a respiratory proton pump to AIF.
Supporting Evidence:
PMID:24914854
The crystal structure of the human AIF (hAIF):NAD(H)-bound dimer revealed one FAD and, unexpectedly, two NAD(H) molecules per protomer.
PMID:24914854
In mitochondria, AIF is present in a monomer-dimer equilibrium that after reduction by NADH gets displaced toward the dimer.
GO:0003954 NADH dehydrogenase activity
EXP
PMID:27178839
Structure/Function Relations in AIFM1 Variants Associated wi...
ACCEPT
Summary: Human AIF variants alter NADH oxidoreductase properties.
Reason: PMID:27178839 explicitly characterizes human V243L, G262S, G308E and G338E proteins and identifies AIF as an NADH-specific oxidoreductase. Full human biochemical experiments in PMID:23217327 corroborate generic acceptor reduction. Retain that chemistry without specifying peroxide formation.
Supporting Evidence:
PMID:27178839
an FAD-containing and NADH-specific oxidoreductase
GO:0003954 NADH dehydrogenase activity
IEA
GO_REF:0000116
ACCEPT
Summary: The Rhea mapping captures the demonstrated NADH-to-acceptor reaction.
Reason: RHEA:11356 matches the exact reaction in UniProt O95831: A + NADH + H(+) = AH2 + NAD(+). Human AIF acceptor-reduction experiments support this generic chemistry; the mapping does not assert membrane proton translocation or a fixed physiological electron acceptor.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
RHEA:11356 SUPPORTS TRANSFER
RHEA:11356 is explicitly present in the human UniProt catalytic activity and fits the experimentally measured generic-acceptor reaction.
Supporting Evidence:
PMID:27178839
an FAD-containing and NADH-specific oxidoreductase
GO:0005515 protein binding
IPI
PMID:16713569
A protein-protein interaction network for human inherited at...
REMOVE
Summary: The ataxia-network binding term adds no resolved AIF molecular function.
Reason: PMID:16713569 reports an interaction network, and GOA/UniProt identify TSC22D4 as the partner. The pair-specific assay was not independently recovered. Removal follows the uninformative generic-protein-binding policy and does not deny that interaction or identify a misattributed gene. No specific AIF activity is inferred from the network association.
GO:0005515 protein binding
IPI
PMID:17094969
Apoptosis-inducing factor (AIF) inhibits protein synthesis b...
MODIFY
Summary: Mature AIF represses translation through EIF3G interaction.
Reason: PMID:17094969 reports yeast two-hybrid, GST pull-down and cellular association, plus inhibition of an in-vitro translation system competitively relieved by EIF3G; inhibition was also observed in cells. GO:0030371 describes this function more informatively than generic binding. The evidence concerns mature AIF in apoptotic/overexpression contexts, not a universal role in basal translation. This refinement rests on the single 2006 study, whose local cache is abstract-only; independent replication was not established in the sources examined.
Proposed replacements: translation repressor activity
Supporting Evidence:
PMID:17094969
we found that mature AIF could inhibit newly-translated protein synthesis and this inhibition was significantly blocked by eIF3g competitively.
GO:0005515 protein binding
IPI
PMID:21364629
Vital function of PRELI and essential requirement of its LEA...
REMOVE
Summary: PRELID1 association does not identify an additional AIF molecular activity.
Reason: Externally inspected PMID:21364629 Figure 6/Methods shows AIF co-immunoprecipitation with WT and LEA-deleted PRELI in human-cell lysates; only WT PRELI preserves mitochondria and prevents AIF redistribution. This supports interaction, but the protective function is assigned to PRELI. Remove the generic AIF binding term without rejecting the association or transferring PRELI functions to AIF.
GO:0005515 protein binding
IPI
PMID:21715506
Comprehensive proteomic analysis of influenza virus polymera...
REMOVE
Summary: Influenza PA association does not resolve a specific AIF molecular function.
Reason: PMID:21715506 identifies human AIFM1 in an influenza-polymerase-associated proteome and proposes possible apoptotic consequences. Preserve the viral PA partner. Remove the uninformative generic binding term without calling the host-virus association false or inferring a viral polymerase or antiviral activity for AIF.
GO:0005515 protein binding
IPI
PMID:22371500
Steroid receptor coactivator-interacting protein (SIP) inhib...
REMOVE
Summary: KANK2/SIP binds and retains AIF in mitochondria.
Reason: Indexed full PMID:22371500 Figure 2 shows reciprocal HeLa co-immunoprecipitation and GST-SIP binding to in-vitro-translated AIF, followed by release/death experiments. The source partner KANK2 is preserved. These data establish regulation of AIF by KANK2 rather than a separate functional binding activity of AIF. Remove only the generic term, retaining the interaction and mitochondrial-retention context.
Supporting Evidence:
PMID:22371500
steroid receptor coactivator-interacting protein (SIP) interacts directly with AIF in mitochondria
GO:0005515 protein binding
IPI
PMID:22371500
Steroid receptor coactivator-interacting protein (SIP) inhib...
REMOVE
Summary: KANK2/SIP binds and retains AIF in mitochondria.
Reason: Indexed full PMID:22371500 Figure 2 shows reciprocal HeLa co-immunoprecipitation and GST-SIP binding to in-vitro-translated AIF, followed by release/death experiments. The source partner the KANK2 isoform Q63ZY3-2 is preserved. These data establish regulation of AIF by KANK2 rather than a separate functional binding activity of AIF. Remove only the generic term, retaining the interaction and mitochondrial-retention context.
Supporting Evidence:
PMID:22371500
steroid receptor coactivator-interacting protein (SIP) interacts directly with AIF in mitochondria
GO:0005515 protein binding
IPI
PMID:26004228
Interaction between AIF and CHCHD4 Regulates Respiratory Cha...
MODIFY
Summary: AIF binds CHCHD4 to anchor its import machinery at the inner membrane.
Reason: Full PMID:26004228 establishes endogenous and recombinant AIF–CHCHD4 association with NADH-sensitive regulation. Human structural/functional evidence in PMID:39806100 resolves membrane anchoring and activation of CHCHD4. The membrane-adaptor activity describes AIF work more accurately than generic binding; CHCHD4 supplies disulfide-transfer chemistry.
Supporting Evidence:
PMID:39806100
NADH allosterically configures AIF to anchor CHCHD4's Ξ²-hairpin and histidine-helix motifs to the inner mitochondrial membrane.
GO:0005515 protein binding
IPI
PMID:33961781
Dual proteome-scale networks reveal cell-specific remodeling...
REMOVE
Summary: The BioPlex KANK2 association is context for an uninformative generic MF.
Reason: PMID:33961781 uses affinity-purification mass spectrometry in human 293T and HCT116 cells; association can include indirect complex contacts. The seeded KANK2 partner is retained and independently supported by PMID:22371500. Remove the generic binding term without calling the pair a false positive or deriving a new activity from AP-MS.
Supporting Evidence:
PMID:22371500
steroid receptor coactivator-interacting protein (SIP) interacts directly with AIF in mitochondria
GO:0005634 nucleus
EXP
PMID:15775970
Export of mitochondrial AIF in response to proapoptotic stim...
ACCEPT
Summary: Processed AIF accumulates in nuclei after death stimulation.
Reason: Full PMID:15775970 imaging/fractionation in HeLa follows endogenous/tagged AIF processing and nuclear redistribution. The nucleus is a functional location for released AIF, not the resting location of the membrane-anchored mitochondrial form.
Supporting Evidence:
PMID:15775970
both the cytoplasmic and nuclear staining patterns of AIF/3xFLAG were observed
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0005634 nucleus
EXP
PMID:17094969
Apoptosis-inducing factor (AIF) inhibits protein synthesis b...
ACCEPT
Summary: The EIF3G study supports nuclear localization of mature AIF.
Reason: PMID:17094969 is the curated UniProt source for AIF nuclear/perinuclear colocalization with EIF3G. Its abstract confirms confocal work, although original images were inaccessible. Retain the correct nuclear location with curator deference and independent full human AIF translocation evidence.
Supporting Evidence:
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0005634 nucleus
EXP
PMID:33168626
The 89-kDa PARP1 cleavage fragment serves as a cytoplasmic P...
ACCEPT
Summary: PAR-bearing PARP1 fragments facilitate AIF nuclear translocation.
Reason: Full PMID:33168626 shows nuclear AIF redistribution in stimulated HeLa cells, with PARP1 cleavage/catalytic mutants and inhibition separating the pathway. Nuclear AIF is a death effector. This experiment involves caspase-activated apoptosis and is not universally caspase-independent.
Supporting Evidence:
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0005634 nucleus
IDA
PMID:23217327
Cowchock syndrome is associated with a mutation in apoptosis...
ACCEPT
Summary: Human patient tissue and cellular experiments support nuclear AIF.
Reason: Full PMID:23217327 shows nuclear AIF in affected human muscle and examines patient/control fibroblast apoptosis; WT and E493V human AIF are tested biochemically. Retain the observed death-associated nuclear location without treating pathological enrichment as normal resting distribution.
Supporting Evidence:
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0005634 nucleus
IEA
GO_REF:0000044
ACCEPT
Summary: The UniProt nuclear mapping represents the released death-associated pool.
Reason: UniProt explicitly records nucleus, supported by human localization studies including PMID:15775970 and PMID:33168626. The mapped compartment is appropriate for nuclear death function; no additional subnuclear precision is imposed.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
UniProtKB-SubCell:SL-0191 SUPPORTS TRANSFER
SL-0191 maps the experimentally supported death-associated nuclear pool.
Supporting Evidence:
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0005634 nucleus
ISS
GO_REF:0000024
ACCEPT
Summary: Conserved nuclear translocation is directly corroborated in human cells.
Reason: The mouse Q9Z0X1 donor has a mitochondrial-to-nuclear death pathway, including PAR-dependent experiments in PMID:21467298. Independent full human PMID:15775970 and PMID:33168626 verify nuclear AIF, supporting the target location without relying exclusively on transfer.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
UniProtKB:Q9Z0X1 SUPPORTS TRANSFER
Mouse Aifm1 translocation is supported by PMID:21467298; independent human microscopy/fractionation confirms the compartment. Mouse constructs expressed in HeLa remain mouse-protein evidence.
Supporting Evidence:
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0005634 nucleus
TAS
PMID:9989411
Molecular characterization of mitochondrial apoptosis-induci...
ACCEPT
Summary: Original characterization describes mitochondrial-to-nuclear movement.
Reason: PMID:9989411 reports nuclear translocation and isolated-nucleus chromatin effects, corroborated by later full human-cell localization. Retain the nucleus as a functional compartment without inferring intrinsic DNA cleavage from nuclear fragmentation.
Supporting Evidence:
PMID:9989411
it is normally confined to mitochondria but translocates to the nucleus when apoptosis is induced.
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0005737 cytoplasm
EXP
PMID:15775970
Export of mitochondrial AIF in response to proapoptotic stim...
KEEP AS NON CORE
Summary: Cytoplasmic AIF is an observed death-associated released pool.
Reason: Full PMID:15775970 separates mitochondrial anchoring, proteolytic detachment and cytoplasmic release before nuclear movement. Retain this source-specific transit/regulated pool; principal mitochondrial and nuclear sites are separately represented.
Supporting Evidence:
PMID:15775970
releasing the processed form into the cytoplasm
GO:0005737 cytoplasm
EXP
PMID:16365034
AIFsh, a novel apoptosis-inducing factor (AIF) pro-apoptotic...
KEEP AS NON CORE
Summary: The AIFsh alternative product is cytosolic before nuclear translocation.
Reason: PMID:16365034 identifies a short C-terminal protein lacking the mitochondrial targeting/redox region; HeLa expression leads to nuclear translocation and death. The cytoplasmic location is valid for this alternative product. Preserve the unqualified source row while explaining isoform scope rather than applying this distribution to all products.
Supporting Evidence:
PMID:16365034
The resulting mRNA encodes a cytosolic protein that corresponds to the C-terminal domain of AIF (amino acids 353-613).
GO:0005737 cytoplasm
EXP
PMID:33168626
The 89-kDa PARP1 cleavage fragment serves as a cytoplasmic P...
KEEP AS NON CORE
Summary: AIF engages a cytoplasmic PAR-carrier pathway during apoptosis.
Reason: Full PMID:33168626 follows PAR-bearing PARP1 fragments and associated AIF in cytoplasm before nuclear redistribution. This is a supported stimulus-dependent pool, not constitutive soluble localization of the normal mitochondrial form.
Supporting Evidence:
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0005737 cytoplasm
IEA
GO_REF:0000044
KEEP AS NON CORE
Summary: The UniProt cytoplasmic pool reflects death-associated redistribution.
Reason: The SL-0086 mapping is supported in UniProt by PMID:15775970 and PMID:33168626 for released AIF. Cytoplasm includes organelles and is not intrinsically incorrect; this source specifically records the regulated extramitochondrial pool, retained as contextual localization.
Propagation Review
Root cause: NO FAILURE NON CORE
Sources checked:
UniProtKB-SubCell:SL-0086 SUPPORTS TRANSFER
The source documents cytoplasmic redistribution after processing; no mapping error or compartment exclusion is asserted.
Supporting Evidence:
PMID:15775970
releasing the processed form into the cytoplasm
GO:0005739 mitochondrion
EXP
PMID:16644725
Identification and characterization of AIFsh2, a mitochondri...
ACCEPT
Summary: AIFsh2 resides in mitochondria and retains redox activity.
Reason: PMID:16644725 identifies mitochondrial localization of the AIFsh2 oxidoreductase-domain product. Preserve organelle resolution; inner-membrane topology and nuclear death activity are not imputed to this product from other isoforms.
Supporting Evidence:
PMID:16644725
The resulting AIFsh2 protein, which localizes in mitochondria, corresponds to the oxidoreductase domain of AIF.
GO:0005739 mitochondrion
HDA
PMID:20833797
Phosphoproteome analysis of functional mitochondria isolated...
ACCEPT
Summary: The human muscle proteomic assignment agrees with direct mitochondrial localization.
Reason: PMID:20833797 analyzes functional mitochondria from resting human muscle. The cached body is partial despite a true full-text flag; the target supplement entry was not independently recovered. Retain the correct mitochondrial assertion with curator deference and independent human topology evidence, without assigning matrix/membrane resolution to this proteomic row.
Supporting Evidence:
PMID:20833797
We performed a phosphoproteomics study of functional mitochondria isolated from human muscle biopsies
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005739 mitochondrion
HTP
PMID:34800366
Quantitative high-confidence human mitochondrial proteome an...
ACCEPT
Summary: MitoCoP supports the mitochondrial assignment.
Reason: PMID:34800366 integrates mitochondrial enrichment, spatial proteomics and import information in human cells. Its HTP assertion agrees with direct AIF experiments. The target supplementary entry was not independently inspected, so retain organelle-level resolution rather than impute a membrane or matrix assay.
Supporting Evidence:
PMID:34800366
We classified >8,000 proteins in mitochondrial preparations of human cells and defined a mitochondrial high-confidence proteome of >1,100 proteins (MitoCoP).
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005739 mitochondrion
IBA
GO_REF:0000033
ACCEPT
Summary: PAINT places conserved mitochondrial function in the AIF lineage.
Reason: PTN002461317 is compatible with human AIF topology and redox/import function. Human O95831 among extant evidence is legitimate experimental grounding, not circular support. No target-specific localization loss is indicated.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
PANTHER:PTN002461317 SUPPORTS TRANSFER
Direct human localization supports the supplied ancestral node-to-target inference. Neither donor count nor target self-evidence diagnoses a propagation failure.
Supporting Evidence:
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005739 mitochondrion
IDA
GO_REF:0000052
ACCEPT
Summary: The immunofluorescence assertion fits established mitochondrial AIF localization.
Reason: GO_REF:0000052 identifies curated immunofluorescence at mitochondrial resolution. The particular image was not independently recovered, but full human fractionation/microscopy establishes the location. Retain the correct broad compartment without claiming antibody-level resolution of subcompartments.
Supporting Evidence:
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005739 mitochondrion
IEA
GO_REF:0000044
ACCEPT
Summary: The UniProt mitochondrial mapping is valid for the mature protein.
Reason: The curated subcellular record and direct topology place mature AIF in mitochondria. Preserve this broad source location rather than overwrite it with separately supported finer membrane/IMS annotations.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
UniProtKB-SubCell:SL-0173 SUPPORTS TRANSFER
SL-0173 is a correct organelle mapping corroborated by direct topology experiments.
Supporting Evidence:
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005739 mitochondrion
TAS
PMID:9989411
Molecular characterization of mitochondrial apoptosis-induci...
ACCEPT
Summary: The first characterization identifies a normally mitochondrial AIF pool.
Reason: PMID:9989411 describes mitochondrial confinement before death-induced nuclear movement. Later human topology confirms the compartment. Retain the TAS at its original organelle resolution.
Supporting Evidence:
PMID:9989411
it is normally confined to mitochondria but translocates to the nucleus when apoptosis is induced.
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005743 mitochondrial inner membrane
EXP
PMID:20111043
A brain-specific isoform of mitochondrial apoptosis-inducing...
ACCEPT
Summary: AIF1 and AIF2 anchor to the mitochondrial inner membrane.
Reason: PMID:20111043 compares AIF1 and alternative-exon-2b AIF2 anchorage and detergent/basic-pH release. This supports inner-membrane location. AIF2 is UniProt O95831-3, not the newer exon-skipping product called AIF3; the unqualified source row is not made isoform-exclusive.
Supporting Evidence:
PMID:20111043
AIF1 and AIF2 are similarly imported to mitochondria in which they anchor to the inner membrane facing the intermembrane space.
GO:0005743 mitochondrial inner membrane
IEA
GO_REF:0000044
ACCEPT
Summary: The membrane mapping captures the mature AIF anchor.
Reason: UniProt and direct topology support an inner-membrane-anchored mature pool with the bulk domain facing the IMS. Retain this location without making the released apoptotic form membrane-bound.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
UniProtKB-SubCell:SL-0168 SUPPORTS TRANSFER
SL-0168 represents the experimentally supported inner-membrane anchor.
Supporting Evidence:
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005743 mitochondrial inner membrane
TAS
PMID:18309324
No death without life: vital functions of apoptotic effector...
ACCEPT
Summary: The review describes inner-membrane-associated AIF.
Reason: Externally inspected Table 2 of PMID:18309324 describes AIF at the inner membrane facing the IMS. Full human topology independently supports the TAS, while the secondary source is not upgraded to a primary experiment.
Supporting Evidence:
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005743 mitochondrial inner membrane
TAS
PMID:39806100
NADH-bound AIF activates the mitochondrial CHCHD4/MIA40 chap...
ACCEPT
Summary: AIF anchors CHCHD4 at the inner mitochondrial membrane.
Reason: Full human structural/functional PMID:39806100 resolves activated AIF–CHCHD4 binding. The established membrane anchor positions the complex. The engineered soluble crystal is not an intact membrane assay; topology is independently supported by PMID:15775970.
Supporting Evidence:
PMID:39806100
NADH allosterically configures AIF to anchor CHCHD4's Ξ²-hairpin and histidine-helix motifs to the inner mitochondrial membrane.
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005758 mitochondrial intermembrane space
EXP
PMID:20111043
A brain-specific isoform of mitochondrial apoptosis-inducing...
ACCEPT
Summary: AIF1 and AIF2 expose their major domains to the IMS.
Reason: PMID:20111043 states that both products anchor to the inner membrane facing the IMS. Preserve their different retention properties and avoid extrapolating the location to cytosolic AIFsh.
Supporting Evidence:
PMID:20111043
AIF1 and AIF2 are similarly imported to mitochondria in which they anchor to the inner membrane facing the intermembrane space.
GO:0005758 mitochondrial intermembrane space
EXP
PMID:24914854
Structural insights into the coenzyme mediated monomer-dimer...
ACCEPT
Summary: The mature redox-active domain occupies the IMS.
Reason: PMID:24914854 supports human AIF coenzyme/redox function, and UniProt links it specifically to IMS localization. Retain this source assertion with curator deference; independent full topology in PMID:15775970 resolves the major domain on the IMS side. The coenzyme crystal itself is not a cellular localization assay.
Supporting Evidence:
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005758 mitochondrial intermembrane space
IDA
PMID:15775970
Export of mitochondrial AIF in response to proapoptotic stim...
ACCEPT
Summary: Topology resolves the mature C-terminal domain in the IMS.
Reason: Full PMID:15775970 uses protease protection, differential permeabilization and extraction to establish inner-membrane anchoring with the major domain in the IMS. Matrix exposure of the short N-terminal segment does not establish a matrix catalytic location.
Supporting Evidence:
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005758 mitochondrial intermembrane space
IDA
PMID:26004228
Interaction between AIF and CHCHD4 Regulates Respiratory Cha...
ACCEPT
Summary: AIF associates with CHCHD4 in the intermembrane compartment.
Reason: Full original PMID:26004228 studies mitochondrial CHCHD4 interaction/import and AIF targeting in human cells and mouse stem cells. IMS AIF agrees with independent human topology. The interaction is not treated as respiratory-complex structural membership.
Supporting Evidence:
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005758 mitochondrial intermembrane space
IEA
GO_REF:0000044
ACCEPT
Summary: The UniProt IMS mapping matches resolved human topology.
Reason: The human location mapping is supported by direct topology and CHCHD4 studies. Retain the IMS location for the mature functional domain while distinguishing release and alternative products.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
UniProtKB-SubCell:SL-0169 SUPPORTS TRANSFER
SL-0169 matches the direct human topology of the major IMS-facing domain.
Supporting Evidence:
PMID:15775970
Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
GO:0005829 cytosol
IEA
GO_REF:0000044
KEEP AS NON CORE
Summary: Cytosol is documented for released AIF, including AIFsh2.
Reason: UniProt maps AIFsh2 to cytosol after calpain/cathepsin-dependent release in proapoptotic conditions (PMID:16644725). Processed full-length AIF also passes through cytoplasm. Preserve this source-specific pool without making it the normal resting location of all products.
Propagation Review
Root cause: NO FAILURE NON CORE
Sources checked:
UniProtKB-SubCell:SL-0091 SUPPORTS TRANSFER
SL-0091 records a real released cytosolic pool with explicit isoform/stimulus scope.
Supporting Evidence:
PMID:15775970
releasing the processed form into the cytoplasm
UniProt:O95831
released from mitochondria to cytosol
GO:0005829 cytosol
ISS
GO_REF:0000024
KEEP AS NON CORE
Summary: The cytosolic transfer fits the released AIF pool.
Reason: The mouse Aifm1 donor and full mouse-protein PAR experiments support cytosolic release. Independent human experiments document cytoplasmic redistribution. Retain conserved extramitochondrial localization as contextual, not constitutive soluble localization of the precursor.
Propagation Review
Root cause: NO FAILURE NON CORE
Sources checked:
UniProtKB:Q9Z0X1 SUPPORTS TRANSFER
Mouse release/translocation is supported in PMID:21467298 and independent human release in PMID:15775970.
Supporting Evidence:
PMID:15775970
releasing the processed form into the cytoplasm
GO:0006915 apoptotic process
IBA
GO_REF:0000033
ACCEPT
Summary: AIF participates as a conserved prodeath effector.
Reason: PTN001115081 is supported by human nuclear redistribution, DNA binding and prodeath experiments. These show effector work rather than necessity alone. AIFsh2 lacks nuclear death activity, so the gene-level assertion does not make every isoform or stimulus pathway equivalent.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
PANTHER:PTN001115081 SUPPORTS TRANSFER
The ancestral assertion agrees with human effector evidence. Target descendant evidence is valid grounding; isoform distinctions do not demonstrate clade-wide loss.
Supporting Evidence:
PMID:23217327
The c.1478A>T (p.Glu493Val) mutation found in the family reported here alters the redox properties of the AIF protein and results in increased cell death via apoptosis
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0006915 apoptotic process
IMP
PMID:23217327
Cowchock syndrome is associated with a mutation in apoptosis...
ACCEPT
Summary: The human E493V study links altered AIF properties to apoptosis.
Reason: Full PMID:23217327 combines genetics, patient tissue/cells and human WT/mutant proteins. Increased nuclear AIF and death in this allele support the apoptotic role alongside independent human WT evidence. Pathological increases are not described as normal rates or universal variant effects.
Supporting Evidence:
PMID:23217327
The c.1478A>T (p.Glu493Val) mutation found in the family reported here alters the redox properties of the AIF protein and results in increased cell death via apoptosis
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0009636 response to toxic substance
IEA
GO_REF:0000107
UNDECIDED
Summary: The rat-to-human toxic substances assertion requires its original experiment and context.
Reason: The supplied donor is rat Aifm1 (UniProtKB:Q9JM53; Ensembl ENSRNOP00000008503), not a paralog. Human AIF has established redox, import and death-effector functions, but the exact donor experiment underlying this toxic substances response was not recovered through the available annotation interfaces. General AIF activities do not establish the particular stimulus, tissue and response mechanism. Retain uncertainty rather than label the donor annotation wrong or infer human conservation solely from orthology.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
UniProtKB:Q9JM53 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for toxic substances remain unresolved. No functional divergence or bad-source conclusion is established.
ensembl:ENSRNOP00000008503 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for toxic substances remain unresolved. No functional divergence or bad-source conclusion is established.
GO:0016174 NAD(P)H oxidase H2O2-forming activity
IBA
GO_REF:0000033
UNDECIDED
Summary: NADH oxidoreduction is established; peroxide-specific chemistry remains unresolved.
Reason: The supplied PTN002461317 node carries a peroxide-forming assertion with legitimate human and mouse descendant evidence. GO:0016174 specifically requires O2 reduction to H2O2. Full PMID:23217327 measures artificial-acceptor reduction and oxygen-dependent flavin reoxidation, which alone does not identify peroxide. The peroxide readout in PMID:39806100 concerns ALR-dependent relay regeneration. Retain uncertainty rather than equate every oxidase assay with this reaction or assert that AIF cannot produce peroxide.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
PANTHER:PTN002461317 UNRESOLVED
Peroxide-specific experiments grounding the PAINT node remain unresolved. Donor count and human self-evidence are not treated as failures, and no redox-activity loss is alleged.
Supporting Evidence:
PMID:27178839
an FAD-containing and NADH-specific oxidoreductase
GO:0016174 NAD(P)H oxidase H2O2-forming activity
IDA
PMID:27178839
Structure/Function Relations in AIFM1 Variants Associated wi...
UNDECIDED
Summary: NADH oxidoreduction is established; peroxide-specific chemistry remains unresolved.
Reason: PMID:27178839 is a real human variant biochemical study, but its full peroxide assay was not recovered. GO:0016174 specifically requires O2 reduction to H2O2. Full PMID:23217327 measures artificial-acceptor reduction and oxygen-dependent flavin reoxidation, which alone does not identify peroxide. The peroxide readout in PMID:39806100 concerns ALR-dependent relay regeneration. Retain uncertainty rather than equate every oxidase assay with this reaction or assert that AIF cannot produce peroxide.
Supporting Evidence:
PMID:27178839
an FAD-containing and NADH-specific oxidoreductase
GO:0016174 NAD(P)H oxidase H2O2-forming activity
TAS
PMID:18309324
No death without life: vital functions of apoptotic effector...
UNDECIDED
Summary: NADH oxidoreduction is established; peroxide-specific chemistry remains unresolved.
Reason: Accessible PMID:18309324 review text describes NADH oxidase/redox biology but does not resolve a peroxide-product assay. GO:0016174 specifically requires O2 reduction to H2O2. Full PMID:23217327 measures artificial-acceptor reduction and oxygen-dependent flavin reoxidation, which alone does not identify peroxide. The peroxide readout in PMID:39806100 concerns ALR-dependent relay regeneration. Retain uncertainty rather than equate every oxidase assay with this reaction or assert that AIF cannot produce peroxide.
Supporting Evidence:
PMID:27178839
an FAD-containing and NADH-specific oxidoreductase
GO:0016491 oxidoreductase activity
IEA
GO_REF:0000002
MODIFY
Summary: Target chemistry refines the broad InterPro redox annotation.
Reason: The IPR023753 mapping is biologically sound; human biochemical evidence resolves NADH-dependent acceptor reduction. Refine the parent to GO:0003954 without claiming every domain-family member uses the same physiological acceptor.
Propagation Review
Root cause: TERM SCOPING PROBLEM
Failure modes: GRANULARITY MISMATCH
Sources checked:
InterPro:IPR023753 SUPPORTS TRANSFER
The broad redox mapping is true; this is target-level specificity refinement, not family misclassification.
Proposed replacements: NADH dehydrogenase activity
Supporting Evidence:
PMID:27178839
an FAD-containing and NADH-specific oxidoreductase
GO:0016651 oxidoreductase activity, acting on NAD(P)H
IDA
PMID:23217327
Cowchock syndrome is associated with a mutation in apoptosis...
MODIFY
Summary: The cited human assays resolve NADH-dependent acceptor reduction.
Reason: Full PMID:23217327 Table 1 measures recombinant human AIF reduction of several acceptors with NADH. The NAD(P)H parent is true; GO:0003954 records the measured donor chemistry without selecting a physiological acceptor or excluding other possible donor activities.
Proposed replacements: NADH dehydrogenase activity
Supporting Evidence:
PMID:23217327
The c.1478A>T (p.Glu493Val) mutation found in the family reported here alters the redox properties of the AIF protein and results in increased cell death via apoptosis
GO:0016651 oxidoreductase activity, acting on NAD(P)H
IDA
PMID:27178839
Structure/Function Relations in AIFM1 Variants Associated wi...
MODIFY
Summary: Human AIF is characterized as an NADH-specific oxidoreductase.
Reason: PMID:27178839 positively identifies this donor specificity, corroborated by full human acceptor-reduction work. Refine the broad NAD(P)H parent to GO:0003954 without imposing unresolved H2O2-product chemistry.
Proposed replacements: NADH dehydrogenase activity
Supporting Evidence:
PMID:27178839
an FAD-containing and NADH-specific oxidoreductase
GO:0033108 mitochondrial respiratory chain complex assembly
IMP
PMID:26004228
Interaction between AIF and CHCHD4 Regulates Respiratory Cha...
UNDECIDED
Summary: Respiratory biogenesis depends on AIF/CHCHD4, but exact assembly-process placement is unresolved.
Reason: Full PMID:26004228 shows AIF–CHCHD4 association, loss of CHCHD4/respiratory proteins after AIF depletion and rescue by engineered AIF-independent CHCHD4 targeting. PMID:39806100 resolves direct import-machinery work. Assembly chaperones need not contact every structural subunit, so lack of complex co-precipitation is not grounds for rejection. The experiments do not decisively separate an assembly-step contribution from upstream import enabling assembly. Preserve the curator IMP assertion with uncertainty at this process boundary.
Supporting Evidence:
PMID:39806100
Regulatory-domain sequestration by NADH-activated AIF directly stimulates chaperone binding and folding
GO:0043065 positive regulation of apoptotic process
IDA
PMID:17094969
Apoptosis-inducing factor (AIF) inhibits protein synthesis b...
ACCEPT
Summary: Mature AIF can amplify apoptosis while repressing translation.
Reason: PMID:17094969 reports EIF3G-dependent translation inhibition and caspase-7 activation after mature-AIF overexpression. This is positive mechanistic support for proapoptotic function, not evidence that every AIF death route is caspase independent or physiological expression always activates death.
Supporting Evidence:
PMID:17094969
mature AIF overexpression specifically resulted in the activation of caspase-7
PMID:17094969
we found that mature AIF could inhibit newly-translated protein synthesis and this inhibition was significantly blocked by eIF3g competitively.
GO:0043065 positive regulation of apoptotic process
TAS
PMID:18309324
No death without life: vital functions of apoptotic effector...
ACCEPT
Summary: Primary effector experiments support the review's positive-apoptosis assertion.
Reason: The original PMID:18309324 AIF section/Table 2 describes released AIF in nuclear chromatin destruction. Human primary studies corroborate nuclear redistribution and prodeath effects. Retain TAS as secondary synthesis, not independent replication or proof of intrinsic AIF nuclease chemistry.
Supporting Evidence:
PMID:23217327
The c.1478A>T (p.Glu493Val) mutation found in the family reported here alters the redox properties of the AIF protein and results in increased cell death via apoptosis
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0043068 positive regulation of programmed cell death
IEA
GO_REF:0000117
ACCEPT
Summary: The broad programmed-death prediction agrees with AIF effector function.
Reason: ARBA00027743 supplies a broad positive-death assertion corroborated by human proapoptotic experiments. Preserve the true parent process rather than infer a particular death mechanism for all products or stimuli.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
ARBA:ARBA00027743 SUPPORTS TRANSFER
Target-level experiments support the broad automated prediction; no rule-scoping failure is demonstrated.
Supporting Evidence:
PMID:23217327
The c.1478A>T (p.Glu493Val) mutation found in the family reported here alters the redox properties of the AIF protein and results in increased cell death via apoptosis
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0043495 protein-membrane adaptor activity
IDA
PMID:39806100
NADH-bound AIF activates the mitochondrial CHCHD4/MIA40 chap...
ACCEPT
Summary: Activated AIF anchors and activates CHCHD4 at the inner membrane.
Reason: Full PMID:39806100 combines human structure, solution measurements, mutagenesis, cellular interaction and substrate-folding assays. AIF sequesters the autoinhibitory CHCHD4 N-terminal segment and supplies a membrane-positioned platform. This is demonstrated adaptor work, not co-IP-only inference or CHCHD4 disulfide catalysis.
Supporting Evidence:
PMID:39806100
NADH allosterically configures AIF to anchor CHCHD4's Ξ²-hairpin and histidine-helix motifs to the inner mitochondrial membrane.
PMID:39806100
Regulatory-domain sequestration by NADH-activated AIF directly stimulates chaperone binding and folding
GO:0043525 positive regulation of neuron apoptotic process
IEA
GO_REF:0000107
UNDECIDED
Summary: The rat-to-human neuron apoptosis assertion requires its original experiment and context.
Reason: The supplied donor is rat Aifm1 (UniProtKB:Q9JM53; Ensembl ENSRNOP00000008503), not a paralog. Human AIF has established redox, import and death-effector functions, but the exact donor experiment underlying this neuron apoptosis response was not recovered through the available annotation interfaces. General AIF activities do not establish the particular stimulus, tissue and response mechanism. Retain uncertainty rather than label the donor annotation wrong or infer human conservation solely from orthology.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
UniProtKB:Q9JM53 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for neuron apoptosis remain unresolved. No functional divergence or bad-source conclusion is established.
ensembl:ENSRNOP00000008503 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for neuron apoptosis remain unresolved. No functional divergence or bad-source conclusion is established.
GO:0045041 protein import into mitochondrial intermembrane space
IMP
PMID:26004228
Interaction between AIF and CHCHD4 Regulates Respiratory Cha...
ACCEPT
Summary: AIF actively supports IMS protein import through CHCHD4.
Reason: Full PMID:26004228 import/rescue experiments establish AIF-dependent CHCHD4 machinery. PMID:39806100 refines this to anchoring and activation of substrate binding/folding; CHCHD4 itself can use its own import motif. AIF supplies active structural/regulatory work in the broad import process, not a transport pore.
Supporting Evidence:
PMID:39806100
Regulatory-domain sequestration by NADH-activated AIF directly stimulates chaperone binding and folding
GO:0046983 protein dimerization activity
IEA
GO_REF:0000002
ACCEPT
Summary: NADH promotes human AIF dimerization and activation.
Reason: IPR029324 agrees with human structure, coenzyme-dependent equilibrium and nondimerizing-mutant data in PMID:24914854, plus the CHCHD4-binding dimer in PMID:39806100. Retain dimerization as part of the redox/adaptor mechanism without asserting obligate dimers under all conditions.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
InterPro:IPR029324 SUPPORTS TRANSFER
Human WT/mutant coenzyme and structural data independently support the domain-based dimerization prediction.
Supporting Evidence:
PMID:24914854
In mitochondria, AIF is present in a monomer-dimer equilibrium that after reduction by NADH gets displaced toward the dimer.
PMID:39806100
NADH allosterically configures AIF to anchor CHCHD4's Ξ²-hairpin and histidine-helix motifs to the inner mitochondrial membrane.
GO:0048471 perinuclear region of cytoplasm
IEA
GO_REF:0000044
KEEP AS NON CORE
Summary: Perinuclear colocalization is a source-linked EIF3G context.
Reason: UniProt attributes nuclear/perinuclear colocalization to PMID:17094969, whose abstract reports confocal experiments. The original image was not recovered; retain the explicitly curated observation with that limitation. This contextual pool does not replace the mitochondrial anchor or imply a new perinuclear organelle.
Propagation Review
Root cause: NO FAILURE NON CORE
Sources checked:
UniProtKB-SubCell:SL-0198 SUPPORTS TRANSFER
SL-0198 maps an explicit source-linked UniProt observation; the unrecovered image is a reassessment limit, not a demonstrated mapping error.
GO:0060545 positive regulation of necroptotic process
ISS
GO_REF:0000024
UNDECIDED
Summary: The necroptosis transfer has an unresolved process-boundary issue.
Reason: MGI links the mouse donor to PAR-dependent AIF experiments including PMID:21467298: purified mouse protein, mouse constructs in HeLa and neuronal rescue. These support PAR-triggered release/death. The live necroptotic-process definition emphasizes RIPK/MLKL signaling and lists parthanatos as related; the experiments do not resolve that mechanism. Preserve uncertainty and the ISS rather than recode mouse constructs as human IDA or equate all regulated necrotic death with this term.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
UniProtKB:Q9Z0X1 UNRESOLVED
Mouse PAR-dependent effector evidence is real; its historical necroptosis assignment and the current RIPK/MLKL boundary need clarification, not a wrong-protein conclusion.
GO:0070059 intrinsic apoptotic signaling pathway in response to endoplasmic reticulum stress
ISS
GO_REF:0000024
KEEP AS NON CORE
Summary: AIF participates in an ER-stress-triggered death pathway in the tested model.
Reason: Original PMID:18056262 Methods identifies full-length human AIF and an N-terminal truncation expressed in mouse embryonic fibroblasts, with thapsigargin, Scythe loss/rescue and redistribution experiments. Retain the conserved contextual role while distinguishing human protein from mouse host. It does not establish basal ER residence or a universal response in human tissues.
Propagation Review
Root cause: NO FAILURE NON CORE
Sources checked:
UniProtKB:Q9Z0X1 SUPPORTS TRANSFER
MGI traces the donor assertion to PMID:18056262; original Methods/Results include human AIF constructs in mouse fibroblasts. Preserve the evidence code and tested context.
GO:0070301 cellular response to hydrogen peroxide
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: AIF participates in death following sustained hydrogen-peroxide exposure.
Reason: The rat source chain remains unresolved, but verified PMID:19418225 reports glucose-oxidase-generated H2O2, AIF knockdown and nuclear AIF-dependent pyknotic death. Human UniProt explicitly links this study to target function. Retain the response as contextual without assigning H2O2 synthesis to AIF or inferring the exact rat tissue/assay.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
UniProtKB:Q9JM53 UNRESOLVED
Independent target evidence supports the judgment, but the exact rat donor experiment remains untraced.
ensembl:ENSRNOP00000008503 UNRESOLVED
Independent target evidence supports the judgment, but the exact rat donor experiment remains untraced.
GO:0070404 NADH binding
IDA
PMID:39806100
NADH-bound AIF activates the mitochondrial CHCHD4/MIA40 chap...
ACCEPT
Summary: NADH binding configures the activated AIF dimer.
Reason: Full PMID:39806100 resolves NADH-dependent human AIF activation and consequences for CHCHD4 binding/folding. PMID:24914854 independently resolves direct NAD(H) binding. Retain ligand recognition as part of the established redox/adaptor mechanism.
Supporting Evidence:
PMID:39806100
NADH allosterically configures AIF to anchor CHCHD4's Ξ²-hairpin and histidine-helix motifs to the inner mitochondrial membrane.
PMID:24914854
The crystal structure of the human AIF (hAIF):NAD(H)-bound dimer revealed one FAD and, unexpectedly, two NAD(H) molecules per protomer.
GO:0071392 cellular response to estradiol stimulus
IEA
GO_REF:0000107
UNDECIDED
Summary: The rat-to-human estradiol assertion requires its original experiment and context.
Reason: The supplied donor is rat Aifm1 (UniProtKB:Q9JM53; Ensembl ENSRNOP00000008503), not a paralog. Human AIF has established redox, import and death-effector functions, but the exact donor experiment underlying this estradiol response was not recovered through the available annotation interfaces. General AIF activities do not establish the particular stimulus, tissue and response mechanism. Retain uncertainty rather than label the donor annotation wrong or infer human conservation solely from orthology.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
UniProtKB:Q9JM53 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for estradiol remain unresolved. No functional divergence or bad-source conclusion is established.
ensembl:ENSRNOP00000008503 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for estradiol remain unresolved. No functional divergence or bad-source conclusion is established.
GO:0071456 cellular response to hypoxia
IEA
GO_REF:0000107
UNDECIDED
Summary: The rat-to-human hypoxia assertion requires its original experiment and context.
Reason: The supplied donor is rat Aifm1 (UniProtKB:Q9JM53; Ensembl ENSRNOP00000008503), not a paralog. Human AIF has established redox, import and death-effector functions, but the exact donor experiment underlying this hypoxia response was not recovered through the available annotation interfaces. General AIF activities do not establish the particular stimulus, tissue and response mechanism. Retain uncertainty rather than label the donor annotation wrong or infer human conservation solely from orthology.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
UniProtKB:Q9JM53 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for hypoxia remain unresolved. No functional divergence or bad-source conclusion is established.
ensembl:ENSRNOP00000008503 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for hypoxia remain unresolved. No functional divergence or bad-source conclusion is established.
GO:0071732 cellular response to nitric oxide
IEA
GO_REF:0000107
UNDECIDED
Summary: The rat-to-human nitric oxide assertion requires its original experiment and context.
Reason: The supplied donor is rat Aifm1 (UniProtKB:Q9JM53; Ensembl ENSRNOP00000008503), not a paralog. Human AIF has established redox, import and death-effector functions, but the exact donor experiment underlying this nitric oxide response was not recovered through the available annotation interfaces. General AIF activities do not establish the particular stimulus, tissue and response mechanism. Retain uncertainty rather than label the donor annotation wrong or infer human conservation solely from orthology.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
UniProtKB:Q9JM53 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for nitric oxide remain unresolved. No functional divergence or bad-source conclusion is established.
ensembl:ENSRNOP00000008503 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for nitric oxide remain unresolved. No functional divergence or bad-source conclusion is established.
GO:0071949 FAD binding
IBA
GO_REF:0000033
ACCEPT
Summary: FAD binding is conserved and directly established in human AIF.
Reason: PTN002461317 fits FAD-containing human structures and biochemistry. Target experimental evidence in the descendant set is valid support. Disease variants can alter properties, but there is no human lineage-wide loss of FAD binding.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
PANTHER:PTN002461317 SUPPORTS TRANSFER
Human structures support inherited FAD binding at the supplied node; target self-evidence is not circular.
Supporting Evidence:
PMID:24914854
The crystal structure of the human AIF (hAIF):NAD(H)-bound dimer revealed one FAD and, unexpectedly, two NAD(H) molecules per protomer.
PMID:27178839
an FAD-containing and NADH-specific oxidoreductase
GO:0071949 FAD binding
IDA
PMID:27178839
Structure/Function Relations in AIFM1 Variants Associated wi...
ACCEPT
Summary: Human WT and variant proteins are FAD-containing oxidoreductases.
Reason: PMID:27178839 explicitly studies FAD-dependent human redox function and variants. PMID:24914854 independently resolves FAD per protomer. Retain cofactor binding without assuming identical occupancy or catalytic efficiency in every variant.
Supporting Evidence:
PMID:27178839
an FAD-containing and NADH-specific oxidoreductase
PMID:24914854
The crystal structure of the human AIF (hAIF):NAD(H)-bound dimer revealed one FAD and, unexpectedly, two NAD(H) molecules per protomer.
GO:0072572 poly-ADP-D-ribose binding
ISS
GO_REF:0000024
ACCEPT
Summary: PAR binding connects AIF release to nuclear death.
Reason: Traced mouse primary PMID:21467298 tests purified WT/mutant AIF and shows disrupted PAR binding impairs release/death. Human-cell PMID:33168626 independently demonstrates PAR-dependent association with PARP1 fragments and redistribution. This supports ISS while preserving the species of the direct biochemical protein and distinguishing polymer recognition from PARP1-polypeptide binding.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
UniProtKB:Q9Z0X1 SUPPORTS TRANSFER
Mouse protein is directly tested in PMID:21467298; human cellular PAR-dependent behavior corroborates conservation. Mouse AIF in HeLa is not human-protein IDA.
Supporting Evidence:
PMID:21467298
WT-AIF caused a PAR mobility shift in a concentration-dependent manner, whereas Pbm-AIF had no effect on PAR mobility
PMID:33168626
In the cytoplasm, AIF binding to PAR attached to the 89-kDa PARP1 fragment facilitated its translocation to the nucleus.
GO:0160203 protein import into the intermembrane space via the disulfide relay system
IBA
GO_REF:0000033
ACCEPT
Summary: The PAINT disulfide-relay inference is directly supported in human AIF.
Reason: PTN002461317 is corroborated by human CHCHD4 activation/import experiments in PMID:39806100. AIF performs anchoring and regulatory-domain sequestration, promoting substrate binding/folding; this is actual work in the process, not necessity alone.
Propagation Review
Root cause: NO FAILURE CORE
Sources checked:
PANTHER:PTN002461317 SUPPORTS TRANSFER
Direct human structure and functional evidence support inherited relay participation through regulatory/scaffold work, not disulfide catalysis by AIF.
Supporting Evidence:
PMID:39806100
Regulatory-domain sequestration by NADH-activated AIF directly stimulates chaperone binding and folding
PMID:39806100
NADH allosterically configures AIF to anchor CHCHD4's Ξ²-hairpin and histidine-helix motifs to the inner mitochondrial membrane.
GO:0160203 protein import into the intermembrane space via the disulfide relay system
IDA
PMID:39806100
NADH-bound AIF activates the mitochondrial CHCHD4/MIA40 chap...
ACCEPT
Summary: AIF directly promotes CHCHD4 substrate recognition and folding.
Reason: Full PMID:39806100 compares NADH alone, AIF with/without NADH and competing CHCHD4 N-terminal peptide. These assays resolve a direct regulatory contribution. CHCHD4 catalyzes substrate disulfide formation and ALR regenerates the relay; their catalytic activities are not assigned to AIF.
Supporting Evidence:
PMID:39806100
Regulatory-domain sequestration by NADH-activated AIF directly stimulates chaperone binding and folding
PMID:39806100
NADH allosterically configures AIF to anchor CHCHD4's Ξ²-hairpin and histidine-helix motifs to the inner mitochondrial membrane.
GO:0160203 protein import into the intermembrane space via the disulfide relay system
TAS
PMID:35994922
AIFM1 beyond cell death: An overview of this OXPHOS-inducing...
ACCEPT
Summary: The review places AIF in CHCHD4-dependent import.
Reason: Full PMID:35994922 synthesizes the import relationship and respiratory consequences; later primary human PMID:39806100 resolves activation. Retain TAS without treating the review as independent experimental replication.
Supporting Evidence:
PMID:35994922
Apoptosis-inducing factor (AIF) is a mitochondrial intermembrane space flavoprotein with diverse functions in cellular physiology.
PMID:39806100
Regulatory-domain sequestration by NADH-activated AIF directly stimulates chaperone binding and folding
GO:1902065 response to L-glutamate
IEA
GO_REF:0000107
UNDECIDED
Summary: The rat-to-human L-glutamate assertion requires its original experiment and context.
Reason: The supplied donor is rat Aifm1 (UniProtKB:Q9JM53; Ensembl ENSRNOP00000008503), not a paralog. Human AIF has established redox, import and death-effector functions, but the exact donor experiment underlying this L-glutamate response was not recovered through the available annotation interfaces. General AIF activities do not establish the particular stimulus, tissue and response mechanism. Retain uncertainty rather than label the donor annotation wrong or infer human conservation solely from orthology.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
UniProtKB:Q9JM53 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for L-glutamate remain unresolved. No functional divergence or bad-source conclusion is established.
ensembl:ENSRNOP00000008503 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for L-glutamate remain unresolved. No functional divergence or bad-source conclusion is established.
GO:1904045 cellular response to aldosterone
IEA
GO_REF:0000107
UNDECIDED
Summary: The rat-to-human aldosterone assertion requires its original experiment and context.
Reason: The supplied donor is rat Aifm1 (UniProtKB:Q9JM53; Ensembl ENSRNOP00000008503), not a paralog. Human AIF has established redox, import and death-effector functions, but the exact donor experiment underlying this aldosterone response was not recovered through the available annotation interfaces. General AIF activities do not establish the particular stimulus, tissue and response mechanism. Retain uncertainty rather than label the donor annotation wrong or infer human conservation solely from orthology.
Propagation Review
Root cause: UNRESOLVED
Sources checked:
UniProtKB:Q9JM53 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for aldosterone remain unresolved. No functional divergence or bad-source conclusion is established.
ensembl:ENSRNOP00000008503 UNRESOLVED
Rat donor identity is verified; the experimental chain and human transfer scope for aldosterone remain unresolved. No functional divergence or bad-source conclusion is established.

Core Functions

Catalyzes NADH-dependent reduction of electron acceptors through its FAD-containing domain in the mitochondrial intermembrane compartment. Human purified-protein assays establish the chemistry, while the dominant physiological acceptor remains unresolved; no proton-pump or peroxide-product activity is implied.

Supporting Evidence:
  • PMID:27178839
    an FAD-containing and NADH-specific oxidoreductase
  • PMID:24914854
    The crystal structure of the human AIF (hAIF):NAD(H)-bound dimer revealed one FAD and, unexpectedly, two NAD(H) molecules per protomer.
  • PMID:15775970
    Topology analysis revealed that mature AIF is a type-I inner membrane protein with the N-terminus exposed to the matrix and the C-terminal portion to the intermembrane space.
  • UniProt:O95831
    Reaction=A + NADH + H(+) = AH2 + NAD(+);

NADH-activated dimeric AIF anchors CHCHD4 at the inner membrane and sequesters its autoinhibitory N-terminal region, enabling substrate recognition and oxidative folding through the disulfide-relay import pathway. AIF supplies structural/regulatory work; CHCHD4 and ALR supply relay chemistry.

Supporting Evidence:
  • PMID:39806100
    NADH allosterically configures AIF to anchor CHCHD4's Ξ²-hairpin and histidine-helix motifs to the inner mitochondrial membrane.
  • PMID:39806100
    Regulatory-domain sequestration by NADH-activated AIF directly stimulates chaperone binding and folding

Released proapoptotic AIF engages chromatin through DNA binding after processing and nuclear translocation. PAR recognition can couple release to redistribution. AIF acts as a death effector; intrinsic DNA cleavage is not assigned, and mechanisms/products depend on stimulus and splice form.

Molecular Function:
DNA binding
Cellular Locations:
Supporting Evidence:
  • UniProt:O95831
    Binds to DNA in a sequence-independent manner
  • PMID:23217327

References

Loading supporting content…

Download this section (compressed HTML)

Suggested Questions for Experts

Q: Which physiological electron acceptors/products are used by human AIF, distinguished from artificial-acceptor assays and ALR-derived peroxide?

Q: Does AIF perform a respiratory-complex assembly step beyond the CHCHD4 import platform, or do import/proteostasis changes explain the assembly phenotype?

Q: How should historical mouse AIF parthanatos evidence be represented relative to current RIPK/MLKL-associated necroptosis terms?

Q: Which human and mouse constructs underlie each purified-protein glutathione-reductase assay in PMID:39979311, and how much does this activity contribute to human mitochondrial glutathione regeneration in vivo?

πŸ“š Additional Documentation

Notes

(AIFM1-notes.md)

Loading supporting content…

Download this section (compressed HTML)

πŸ“„ View Raw YAML

Loading supporting content…

Download this section (compressed HTML)