GO_REF:0000002
Gene Ontology annotation through association of InterPro records with GO terms
GO_REF:0000024
Manual transfer of experimentally-verified manual GO annotation data to orthologs by curator judgment of sequence similarity
GO_REF:0000033
Annotation inferences using phylogenetic trees
GO_REF:0000052
Gene Ontology annotation based on curation of immunofluorescence data
GO_REF:0000107
Automatic transfer of experimentally verified manual GO annotation data to orthologs using Ensembl Compara
GO_REF:0000120
Combined Automated Annotation using Multiple IEA Methods
PMID:10395799
Identification and characterization of AFG3L2, a novel paraplegin-related gene.
PMID:14623864
Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia.
PMID:19748354
An intersubunit signaling network coordinates ATP hydrolysis by m-AAA proteases.
PMID:22354088
Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment.
PMID:26387735
SPG7 Is an Essential and Conserved Component of the Mitochondrial Permeability Transition Pore.
PMID:26504172
Quality control of mitochondrial protein synthesis is required for membrane integrity and cell fitness.
PMID:27642048
The m-AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria.
PMID:28396416
Proteolytic control of the mitochondrial calcium uniporter complex.
PMID:29545505
m-AAA and i-AAA complexes coordinate to regulate OMA1, the stress-activated supervisor of mitochondrial dynamics.
PMID:29932645
Dissecting Substrate Specificities of the Mitochondrial AFG3L2 Protease.
PMID:30683687
Mitochondrial stress response triggered by defects in protein synthesis quality control.
PMID:31327635
Unique Structural Features of the Mitochondrial AAA+ Protease AFG3L2 Reveal the Molecular Basis for Activity in Health and Disease.
PMID:32814053
Interactome Mapping Provides a Network of Neurodegenerative Disease Proteins and Uncovers Widespread Protein Aggregation in Affected Brains.
PMID:34718584
Translation of MT-ATP6 pathogenic variants reveals distinct regulatory consequences from the co-translational quality control of mitochondrial protein synthesis.
PMID:34800366
Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context.
PMID:35912435
Regulation of mitochondrial proteostasis by the proton gradient.
PMID:37917749
Autoregulatory control of mitochondrial glutathione homeostasis.
PMID:38157846
Dual regulation of SLC25A39 by AFG3L2 and iron controls mitochondrial glutathione homeostasis.
PMID:41075794
SLC25A45 is required for mitochondrial uptake of methylated amino acids and de novo carnitine biosynthesis.
Reactome:R-HSA-8949649
PMPCA:PMPCB cleaves the transit peptide of proSMDT1 (proEMRE)
Reactome:R-HSA-8949659
AFG3L2 (m-AAA protease) degrades SMDT1 that is not assembled in MCU
Reactome:R-HSA-8949661
C2orf47:AFG3L2 binds the transit peptide of SMDT1
Reactome:R-HSA-9838627
AFG3L2 binds mitochondrial inner membrane proteins
Reactome:R-HSA-9839053
AFG3L2:SPG7 binds SMDT1 (EMRE)
Reactome:R-HSA-9839059
AFG3L2:SPG7 degrades SMDT1 (EMRE)
Reactome:R-HSA-9839105
AFG3L2 degrades mitochondrial matrix proteins
Reactome:R-HSA-9839113
AFG3L2 degrades mitochondrial inner membrane proteins
Reactome:R-HSA-9839149
AFG3L2 binds mitochondrial matrix proteins
file:human/AFG3L2/AFG3L2-uniprot.txt
UniProtKB record for human AFG3L2 (Q9Y4W6)
file:human/AFG3L2/AFG3L2-deep-research-falcon.md
Falcon deep research report for human AFG3L2
file:human/AFG3L2/AFG3L2-notes.md
AFG3L2 curator notes for Proteostasis PN review
file:projects/PROTEOSTASIS/reports/pn_projection/pn_projected_annotations.tsv
Proteostasis Network projected GO annotations report
file:projects/PROTEOSTASIS/mappings/mitochondrial_proteostasis.yaml
Proteostasis Network mitochondrial proteostasis mapping
PMID:19656850
Autocatalytic processing of m-AAA protease subunits in mitochondria.
PMID:30252181
Concurrent AFG3L2 and SPG7 mutations associated with syndromic parkinsonism and optic atrophy with aberrant OPA1 processing and mitochondrial network fragmentation.
PMID:36301938
Molecular Determinants of Mitochondrial Shape and Function and Their Role in Glaucoma.
PMID:37804316
Sustained OMA1-mediated integrated stress response is beneficial for spastic ataxia type 5.
PMID:38012514
Multifaceted Roles of AFG3L2, a Mitochondrial ATPase in Relation to Neurological Disorders.
PMID:38390227
Autosomal recessive cerebellar ataxias: a diagnostic classification approach according to ocular features.