About   Help   FAQ
Sequence Detail
ID/Version
Q920A7 Q9CZN2 Q6PGJ7 Q3THK2 (UniProt | EBI) Last sequence update: 2008-02-26
Last annotation update: 2025-06-18
Sequence
description
from provider
RecName: Full=Mitochondrial inner membrane m-AAA protease component AFG3L1 {ECO:0000305}; EC=3.4.24.- {ECO:0000269|PubMed:11549317}; EC=3.6.-.- {ECO:0000250|UniProtKB:Q9Y4W6};AltName: Full=AFG3-like protein 1;Flags: Precursor;
Provider SWISS-PROT
Sequence
Polypeptide 789 aa
For this sequence
Source
Organism mouse
See UniProt | EBI for source
Annotated genes and markers Follow the symbol links to get more information on the GO terms, expression assays, orthologs, phenotypic alleles, and other information for the genes or markers below.
Type Symbol Name GO Terms Expression
Assays
Orthologs Phenotypic
Alleles
Gene Afg3l1 AFG3-like AAA ATPase 1 45 96 3 5
Sequence references in MGI J:71129 Kremmidiotis G, et al., Molecular and functional analyses of the human and mouse genes encoding afg3l1, a mitochondrial metalloprotease homologous to the human spastic paraplegia protein. Genomics. 2001 Aug;76(1-3):58-65
J:99680 The FANTOM Consortium and RIKEN Genome Exploration Research Group and Genome Science Group (Genome Network Project Core Group), The Transcriptional Landscape of the Mammalian Genome. Science. 2005;309(5740):1559-1563
J:117691 Koppen M, et al., Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia. Mol Cell Biol. 2007 Jan;27(2):758-67
J:164484 Koppen M, et al., Autocatalytic processing of m-AAA protease subunits in mitochondria. Mol Biol Cell. 2009 Oct;20(19):4216-24
J:237410 Wang S, et al., The Mitochondrial m-AAA Protease Prevents Demyelination and Hair Greying. PLoS Genet. 2016 Dec;12(12):e1006463
J:292518 Huttlin EL, et al., A tissue-specific atlas of mouse protein phosphorylation and expression. Cell. 2010 Dec 23;143(7):1174-89

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources
Funding Information
Warranty Disclaimer, Privacy Notice, Licensing, & Copyright
Send questions and comments to User Support.
last database update
09/30/2025
MGI 6.24
The Jackson Laboratory