FAF2

UniProt ID: Q96CS3
Organism: Homo sapiens
Review Status: COMPLETE
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Gene Description

FAF2 (also known as UBXD8, ETEA, UBXN3B) is a 445-residue, membrane-anchored cofactor of the AAA-ATPase p97/VCP. It contains an N-terminal UBA domain that binds ubiquitin conjugates, a central UAS (thioredoxin-like) domain implicated in unsaturated fatty-acid sensing, a coiled-coil region, and a C-terminal UBX domain that docks onto p97/VCP. By bridging ubiquitinated client proteins to the p97/VCP segregase, FAF2 functions as a substrate-recruiting adaptor at the endoplasmic reticulum membrane and on lipid droplets. At the ER it is a component of the SEL1L/HRD1-associated ER-associated degradation (ERAD) machinery, where it promotes the dislocation/retrotranslocation of misfolded ER proteins to the cytosol for proteasomal degradation. On lipid droplets, FAF2 recruits p97/VCP and binds directly to the lipase PNPLA2/ATGL, inhibiting it by displacing its coactivator ABHD5/CGI-58, thereby restraining triacylglycerol lipolysis and increasing lipid-droplet size; its partitioning between the ER and lipid droplets is controlled by the ER-resident rhomboid pseudoprotease UBAC2. FAF2 also links lipid metabolism through p97-dependent dislocation of Insig-1 (SREBP regulation) and, upon heat stress, binds ubiquitinated G3BP1 to recruit p97/VCP and drive stress-granule disassembly. FAF2 is broadly expressed.

Existing Annotations Review

GO Term Evidence Action Reason
GO:0036503 ERAD pathway
IBA
GO_REF:0000033
ACCEPT
Summary: Phylogenetic inference of FAF2's role in ERAD, consistent with strong experimental evidence that FAF2/UBXD8 is part of the ER dislocation machinery.
Reason: Core biological process; FAF2 is a p97/VCP cofactor in ER-associated degradation, supported experimentally.
Supporting Evidence:
PMID:18711132
We identified AUP1, UBXD8, UBC6e, and OS9 as functionally important
GO:0005737 cytoplasm
IEA
GO_REF:0000044
KEEP AS NON CORE
Summary: Electronic transfer of cytoplasmic localization from the UniProt subcellular location; FAF2 is a membrane protein of the ER/lipid droplet, which are cytoplasmic structures.
Reason: Correct but generic; the specific ER and lipid-droplet compartments capture FAF2's site of action more informatively.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0005783 endoplasmic reticulum
IEA
GO_REF:0000044
ACCEPT
Summary: Electronic assignment of ER localization, consistent with direct experimental evidence that FAF2 is an ER membrane protein.
Reason: Correct core compartment; redundant with IDA evidence.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Endoplasmic reticulum
GO:0005811 lipid droplet
IEA
GO_REF:0000044
ACCEPT
Summary: Electronic assignment of lipid-droplet localization, consistent with direct experimental and proteomic evidence.
Reason: Correct core compartment; FAF2 partitions between ER and lipid droplets.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Lipid droplet
GO:0010498 proteasomal protein catabolic process
IEA
GO_REF:0000117
KEEP AS NON CORE
Summary: ARBA machine-learning assignment of proteasomal protein catabolism, a generic parent of FAF2's specific ERAD/proteasome-mediated degradation role.
Reason: Correct but generic; GO:0036503 (ERAD pathway) better captures the specific function.
Supporting Evidence:
PMID:18711132
dislocation of misfolded
GO:0005515 protein binding
IPI
PMID:18711132
SEL1L nucleates a protein complex required for dislocation o...
KEEP AS NON CORE
Summary: IntAct capture of FAF2 protein interaction (VCP). The bare protein binding term is uninformative; the VCP interaction is better captured by ubiquitin protein ligase binding and the VCP complex annotations.
Reason: Records a real interaction but bare protein binding is uninformative per curation guidelines.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Q96CS3; P55072: VCP
GO:0005515 protein binding
IPI
PMID:18775313
UBXD7 binds multiple ubiquitin ligases and implicates p97 in...
KEEP AS NON CORE
Summary: IntAct capture of FAF2-VCP interaction from the UBX-domain/p97 cofactor study. Bare protein binding is uninformative; better represented by the specific p97/ubiquitin-binding terms.
Reason: Real p97 interaction but uninformative GO term.
Supporting Evidence:
PMID:18775313
p97 assembles with all of the 13 mammalian UBX-domain proteins
GO:0005515 protein binding
IPI
PMID:22119785
Defining human ERAD networks through an integrative mapping ...
KEEP AS NON CORE
Summary: IntAct interaction captured in an ERAD network mapping study (partners VCP and UBAC2). Bare protein binding is uninformative.
Reason: Real ERAD-network interactions but uninformative GO term.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Q96CS3; Q8NBM4: UBAC2
GO:0005515 protein binding
IPI
PMID:28514442
Architecture of the human interactome defines protein commun...
KEEP AS NON CORE
Summary: High-throughput interactome capture (UBAC2). Bare protein binding is uninformative.
Reason: Real interaction from a large-scale interactome but uninformative GO term.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Q96CS3; Q8NBM4: UBAC2
GO:0005515 protein binding
IPI
PMID:29997244
LuTHy: a double-readout bioluminescence-based two-hybrid tec...
KEEP AS NON CORE
Summary: LuTHy two-hybrid capture of FAF2-VCP interaction. Bare protein binding is uninformative.
Reason: Real interaction but uninformative GO term.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Q96CS3; P55072: VCP
GO:0005515 protein binding
IPI
PMID:35044719
Proteome-scale mapping of binding sites in the unstructured ...
KEEP AS NON CORE
Summary: Proteome-scale binding-site mapping capturing a FAF2 interaction (VASP). Bare protein binding is uninformative.
Reason: Real interaction but uninformative GO term.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Q96CS3; P50552: VASP
GO:0005515 protein binding
IPI
PMID:35271311
OpenCell: Endogenous tagging for the cartography of human ce...
KEEP AS NON CORE
Summary: OpenCell endogenous-tagging interactome capture (VCP). Bare protein binding is uninformative.
Reason: Real interaction but uninformative GO term.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Q96CS3; P55072: VCP
GO:0005515 protein binding
IPI
PMID:37776851
Analysis of proteome-wide degradation dynamics in ALS SOD1 i...
KEEP AS NON CORE
Summary: Interaction captured in an ALS SOD1 degradation-dynamics study implicating VCP homeostasis. Bare protein binding is uninformative.
Reason: Real interaction but uninformative GO term.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Q96CS3; P55072: VCP
GO:0005515 protein binding
IPI
PMID:40205054
Multimodal cell maps as a foundation for structural and func...
KEEP AS NON CORE
Summary: Multimodal cell-map interactome capture (UBAC2). Bare protein binding is uninformative.
Reason: Real interaction but uninformative GO term.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Q96CS3; Q8NBM4: UBAC2
GO:0043161 proteasome-mediated ubiquitin-dependent protein catabolic process
IEA
GO_REF:0000107
KEEP AS NON CORE
Summary: Orthology-based assignment of proteasome-mediated ubiquitin-dependent catabolism, consistent with FAF2's adaptor role in delivering ubiquitinated substrates to p97 for proteasomal degradation.
Reason: Correct but a generic parent of the specific ERAD process; retained as non-core.
Supporting Evidence:
PMID:18711132
dislocation of misfolded
GO:0034098 VCP-NPL4-UFD1 AAA ATPase complex
NAS
PMID:28819009
The AAA+ ATPase p97, a cellular multitool.
ACCEPT
Summary: FAF2 is a substrate-recruiting cofactor that assembles with the core p97/VCP-NPL4-UFD1 segregase (ComplexPortal CPX-8104, VCP-NPL4-UFD1-FAF2 complex).
Reason: Correct; FAF2 forms a defined p97/VCP cofactor complex, also supported by direct evidence (PMID:18775313).
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
VCP-NPL4-UFD1-FAF2 AAA ATPase complex
GO:0036503 ERAD pathway
NAS
PMID:28819009
The AAA+ ATPase p97, a cellular multitool.
ACCEPT
Summary: ComplexPortal assertion of FAF2's ERAD role as a p97/VCP cofactor, consistent with experimental evidence.
Reason: Core biological process; redundant with experimental IMP/IBA ERAD annotations.
Supporting Evidence:
PMID:18711132
We identified AUP1, UBXD8, UBC6e, and OS9 as functionally important
GO:1904949 ATPase complex
NAS
PMID:28819009
The AAA+ ATPase p97, a cellular multitool.
KEEP AS NON CORE
Summary: Generic parent term for the p97/VCP ATPase complex to which FAF2 binds.
Reason: Correct but generic; the specific VCP-NPL4-UFD1 complex term is more informative.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
VCP-NPL4-UFD1-FAF2 AAA ATPase complex
GO:0005783 endoplasmic reticulum
IDA
GO_REF:0000052
ACCEPT
Summary: Direct immunofluorescence (HPA) evidence for ER localization, consistent with FAF2's role as an ER membrane protein.
Reason: Core compartment; FAF2 acts at the ER membrane.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Endoplasmic reticulum
GO:0005811 lipid droplet
IDA
GO_REF:0000052
ACCEPT
Summary: Direct immunofluorescence (HPA) evidence for lipid-droplet localization, consistent with FAF2's lipid-droplet function.
Reason: Core compartment; FAF2 traffics to lipid droplets to recruit p97/VCP.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Lipid droplet
GO:0005783 endoplasmic reticulum
IDA
PMID:34739333
Ubiquitination of G3BP1 mediates stress granule disassembly ...
ACCEPT
Summary: FAF2 is active at the ER, where it engages ubiquitinated G3BP1 and recruits p97/VCP during heat-stress stress-granule disassembly.
Reason: Correct site of action; directly demonstrated.
Supporting Evidence:
PMID:34739333
the endoplasmic reticulum–associated protein FAF2
GO:0030674 protein-macromolecule adaptor activity
IDA
PMID:34739333
Ubiquitination of G3BP1 mediates stress granule disassembly ...
ACCEPT
Summary: FAF2 acts as an adaptor that bridges ubiquitinated G3BP1 to the p97/VCP segregase, an informative molecular function reflecting its general p97-cofactor adaptor role.
Reason: Core molecular function; FAF2 couples ubiquitinated clients to p97/VCP.
Supporting Evidence:
PMID:34739333
ubiquitinated G3BP1 interacted with the endoplasmic reticulum–associated protein FAF2, which engaged the ubiquitin-dependent segregase p97/VCP
GO:0035617 stress granule disassembly
IDA
PMID:34739333
Ubiquitination of G3BP1 mediates stress granule disassembly ...
ACCEPT
Summary: FAF2 promotes heat-stress-induced stress-granule disassembly by recruiting p97/VCP to ubiquitinated G3BP1, extracting it from the granule.
Reason: Directly demonstrated biological process; a distinct (non-ERAD) role of FAF2 as a p97 adaptor.
Supporting Evidence:
PMID:34739333
targeting of G3BP1 weakened the stress granule–specific interaction network
GO:0005515 protein binding
IPI
PMID:31073040
LMBR1L regulates lymphopoiesis through Wnt/Ξ²-catenin signali...
KEEP AS NON CORE
Summary: IPI capture of FAF2-LMBR1L interaction. Bare protein binding is uninformative.
Reason: Real interaction (LMBR1L) but uninformative GO term.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Interacts with LMBR1L
GO:0005576 extracellular region
TAS
Reactome:R-HSA-6798751
MARK AS OVER ANNOTATED
Summary: Reactome neutrophil-degranulation pathway annotation placing FAF2 in the extracellular region via azurophil-granule exocytosis. This derives from bulk granule proteomics and does not reflect FAF2's characterized ER/lipid-droplet membrane function.
Reason: FAF2 is an integral ER/lipid-droplet membrane protein; extracellular localization is an over-annotation from neutrophil-granule proteomics, not its biological site of action.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0035578 azurophil granule lumen
TAS
Reactome:R-HSA-6798751
MARK AS OVER ANNOTATED
Summary: Reactome neutrophil-degranulation annotation placing FAF2 in the azurophil granule lumen, from bulk neutrophil-granule proteomics; inconsistent with FAF2 being an integral cytoplasmic-facing ER/lipid-droplet membrane protein.
Reason: Over-annotation from granule proteomics; not the characterized ER/lipid-droplet function.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0030970 retrograde protein transport, ER to cytosol
IMP
PMID:25660456
Identification of ERAD components essential for dislocation ...
ACCEPT
Summary: FAF2/UBXD8 was experimentally required for dislocation (retrotranslocation) of the NHK alpha-1-antitrypsin ERAD substrate from the ER lumen to the cytosol.
Reason: Directly supported (IMP); captures the dislocation/retrotranslocation step of ERAD in which FAF2 participates.
Supporting Evidence:
PMID:25660456
dislocation, also known as retrotranslocation, of those unwanted proteins from
GO:0005515 protein binding
IPI
PMID:24215460
Pathogenic mutation of UBQLN2 impairs its interaction with U...
KEEP AS NON CORE
Summary: IPI capture of FAF2-UBQLN2 interaction. Bare protein binding is uninformative; the functional cooperation in ERAD is captured by the ERAD process annotation.
Reason: Real interaction (UBQLN2) but uninformative GO term.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Interacts (via N-terminus) with UBQLN2
GO:0036503 ERAD pathway
IMP
PMID:24215460
Pathogenic mutation of UBQLN2 impairs its interaction with U...
ACCEPT
Summary: Loss/disruption of FAF2/UBXD8 interaction with UBQLN2 impaired ERAD, demonstrating FAF2's functional requirement in the pathway.
Reason: Core biological process with direct mutant/perturbation (IMP) support.
Supporting Evidence:
PMID:24215460
disrupts endoplasmic reticulum-associated protein degradation
GO:0005783 endoplasmic reticulum
IDA
PMID:23297223
Spatial regulation of UBXD8 and p97/VCP controls ATGL-mediat...
ACCEPT
Summary: Direct evidence for ER localization of FAF2/UBXD8, the compartment from which it traffics to lipid droplets.
Reason: Core compartment; directly demonstrated.
Supporting Evidence:
PMID:23297223
UBXD8 with the ER-resident rhomboid pseudoprotease UBAC2
GO:0005811 lipid droplet
IDA
PMID:23297223
Spatial regulation of UBXD8 and p97/VCP controls ATGL-mediat...
ACCEPT
Summary: Direct evidence that FAF2/UBXD8 localizes to lipid droplets, where it recruits p97/VCP and regulates ATGL.
Reason: Core compartment; directly demonstrated.
Supporting Evidence:
PMID:23297223
UBXD8-mediated recruitment of p97/VCP to LDs
GO:0034389 lipid droplet organization
IMP
PMID:23297223
Spatial regulation of UBXD8 and p97/VCP controls ATGL-mediat...
ACCEPT
Summary: FAF2/UBXD8 controls lipid-droplet size by recruiting p97/VCP and inhibiting ATGL-mediated triacylglycerol hydrolysis, regulating lipid-droplet organization.
Reason: Directly supported biological process; FAF2 governs lipid-droplet dynamics via ATGL regulation.
Supporting Evidence:
PMID:23297223
recruitment of p97/VCP to LDs increases
GO:0035473 lipase binding
IDA
PMID:23297223
Spatial regulation of UBXD8 and p97/VCP controls ATGL-mediat...
ACCEPT
Summary: FAF2/UBXD8 binds directly to the lipase ATGL/PNPLA2, a specific and informative molecular function.
Reason: Directly demonstrated specific binding; supports the lipase-inhibitor and lipid-droplet roles.
Supporting Evidence:
PMID:23297223
UBXD8 binds directly to ATGL
GO:0055102 lipase inhibitor activity
IDA
PMID:23297223
Spatial regulation of UBXD8 and p97/VCP controls ATGL-mediat...
ACCEPT
Summary: FAF2/UBXD8 inhibits ATGL/PNPLA2 lipase activity by promoting dissociation of its coactivator CGI-58/ABHD5, a specific molecular function.
Reason: Directly demonstrated lipase-inhibitor activity; a defining lipid-metabolic function of FAF2.
Supporting Evidence:
PMID:23297223
promotes dissociation of its endogenous coactivator, CGI-58
GO:0005811 lipid droplet
IDA
PMID:14741744
Identification of major proteins in the lipid droplet-enrich...
ACCEPT
Summary: Lipid-droplet proteomic identification of FAF2/UBXD8 in the LD-enriched fraction of HuH7 hepatocytes, corroborating its lipid-droplet localization.
Reason: Independent IDA support for the lipid-droplet compartment.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Lipid droplet
GO:0031625 ubiquitin protein ligase binding
IDA
PMID:18775313
UBXD7 binds multiple ubiquitin ligases and implicates p97 in...
ACCEPT
Summary: As a UBX-domain p97 cofactor, FAF2/UBXD8 binds E3 ubiquitin ligases, linking ubiquitinated substrates to p97/VCP. This is an informative molecular function reflecting its adaptor role.
Reason: Directly demonstrated; UBX-domain p97 cofactors that bind ubiquitin conjugates also interact with E3 ubiquitin ligases.
Supporting Evidence:
PMID:18775313
also interact with dozens of E3
GO:0034098 VCP-NPL4-UFD1 AAA ATPase complex
IDA
PMID:18775313
UBXD7 binds multiple ubiquitin ligases and implicates p97 in...
ACCEPT
Summary: FAF2/UBXD8 directly assembles with the p97/VCP-NPL4-UFD1 segregase as a substrate-recruiting cofactor.
Reason: Core complex membership with direct (IDA) support.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
VCP-NPL4-UFD1-FAF2 AAA ATPase complex
GO:0043130 ubiquitin binding
IDA
PMID:18775313
UBXD7 binds multiple ubiquitin ligases and implicates p97 in...
ACCEPT
Summary: FAF2/UBXD8 binds ubiquitin/ubiquitin conjugates through its N-terminal UBA domain, the molecular basis for recognizing ubiquitinated p97 clients.
Reason: Core informative molecular function; the UBA domain binds polyubiquitinated substrates for delivery to p97/VCP.
Supporting Evidence:
PMID:18775313
proteins that bind ubiquitin conjugates
GO:0005515 protein binding
IPI
PMID:19818707
The otubain YOD1 is a deubiquitinating enzyme that associate...
KEEP AS NON CORE
Summary: IPI capture of FAF2-YOD1 interaction (a p97-associated deubiquitinase in ER dislocation). Bare protein binding is uninformative.
Reason: Real interaction (YOD1) but uninformative GO term.
Supporting Evidence:
file:human/FAF2/FAF2-uniprot.txt
Interacts with YOD1

Core Functions

Ubiquitin-binding adaptor (via its N-terminal UBA domain) that recognizes polyubiquitinated client proteins and, through its C-terminal UBX domain, recruits the p97/VCP segregase, coupling ubiquitinated substrates to ATP-driven extraction.

Molecular Function:
ubiquitin binding
Supporting Evidence:
  • PMID:18775313
    proteins that bind ubiquitin conjugates
  • PMID:34739333
    ubiquitinated G3BP1 interacted with the endoplasmic reticulum–associated protein FAF2, which engaged the ubiquitin-dependent segregase p97/VCP

ER-membrane p97/VCP cofactor in the SEL1L/HRD1 ER-associated degradation machinery that promotes dislocation/retrotranslocation of misfolded ER proteins to the cytosol for proteasomal degradation.

Supporting Evidence:
  • PMID:18711132
    We identified AUP1, UBXD8, UBC6e, and OS9 as functionally important
  • PMID:25660456
    dislocation, also known as retrotranslocation, of those unwanted proteins from

Lipid-droplet regulator that recruits p97/VCP and binds directly to the lipase ATGL/PNPLA2, inhibiting it by displacing the coactivator CGI-58/ABHD5, thereby restraining triacylglycerol lipolysis and controlling lipid-droplet size.

Molecular Function:
lipase inhibitor activity
Directly Involved In:
Cellular Locations:
Supporting Evidence:

References

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Suggested Questions for Experts

Q: To what extent does FAF2's UAS-domain unsaturated-fatty-acid sensing directly gate its ER-to-lipid-droplet partitioning and ATGL inhibition under different metabolic states?

Q: Is FAF2's role in p97-dependent dislocation of Insig-1 (SREBP regulation) mechanistically separable from its general ERAD substrate-extraction adaptor activity?

Suggested Experiments

Experiment: Reconstitute p97/VCP-dependent extraction of a defined ubiquitinated ERAD substrate with purified FAF2, UFD1-NPL4 and p97 to test whether FAF2 is sufficient as the substrate-recruiting cofactor and to map its UBA-dependence.

Experiment: Use proximity labeling (BioID/TurboID) of FAF2 in cells shifted between fed and lipolytic states to define how the ER versus lipid-droplet interactomes (including UBAC2, ATGL, CGI-58) change with metabolic condition.

Experiment: Quantitative degradation assays of endogenous ERAD substrates (e.g. NHK alpha-1-antitrypsin) and lipid-droplet ATGL turnover in FAF2 UBA-domain and UBX-domain point mutants to dissociate the ubiquitin-binding from the p97-recruiting contributions.

Deep Research

Falcon

(FAF2-deep-research-falcon.md)

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πŸ“š Additional Documentation

Notes

(FAF2-notes.md)

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Pn Notes

(FAF2-pn-notes.md)

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