Afg3l1
entrez
114896
canonical
uniprot
Q920A7
canonical
ensembl
ENSMUSG00000031967
resolver
ensp
ENSMUSP00000001520
resolver
ensp
ENSMUSP00000351230
resolver
genesymbol
Afg3l1
resolver
uniprot
Q3THK2
resolver
uniprot
Q6PGJ7
resolver
uniprot
Q9CZN2
resolver
uniprot_entry
AFG31_MOUSE
resolver
uniprot_sec
Q3THK2
resolver
uniprot_sec
Q6PGJ7
resolver
uniprot_sec
Q9CZN2
resolver
amino_acid_sequence
MLLRLVGAAGSRALAWPFSKLWRCGGCAGSGGTVWSSVRACGIALQGHLGRCSQQLALQGKLTSFSPRLYSKPPRGFEKFFKNKKNRKSASPGNSVPPKKEPKNAGPGGDGGNRGGKGDDFPWWKRMQKGEFPWDDKDFRSLAVLGAGVAAGFLYFYFRDPGKEITWKHFVQYYLARGLVDRLEVVNKQFVRVIPVPGTTSERFVWFNIGSVDTFERNLESAQWELGIEPTNQAAVVYTTESDGSFLRSLVPTLVLVSILLYAMRRGPMGTGRGGRGGGLFSVGETTAKILKNNIDVRFADVAGCEEAKLEIMEFVNFLKNPKQYQDLGAKIPKGAMLTGPPGTGKTLLAKATAGEANVPFITVNGSEFLEMFVGVGPARVRDMFAMARKHAPCILFIDEIDAIGRKRGRGHLGGQSEQENTLNQMLVEMDGFNSSTNVVVLAGTNRPDILDPALTRPGRFDRQIYIGPPDIKGRSSIFKVHLRPLKLDGSLSKDALSRKLAALTPGFTGADISNVCNEAALIAARHLSPSVQERHFEQAIERVIGGLEKKTQVLQPSEKTTVAYHEAGHAVVGWFLEHADPLLKVSIIPRGKGLGYAQYLPREQFLYTREQLFDRMCMMLGGRVAEQLFFGQITTGAQDDLRKVTQSAYAQIVQFGMSEKLGQVSFDFPRQGETMVEKPYSEATAQLIDEEVRCLVRSAYNRTLELLTQCREQVEKVGRRLLEKEVLEKADMIELLGPRPFAEKSTYEEFVEGTGSLEEDTSLPEGLKDWNKGREEGGTERGLQESPV
uniprot
proteins
ec_number
3.4.24.-
uniprot
proteins
ec_number
3.6.-.-
uniprot
proteins
function
FUNCTION: Catalytic component of the m-AAA protease, a protease that plays a key role in proteostasis of inner mitochondrial membrane proteins, and which is essential for axonal and neuron development (PubMed:11549317, PubMed:19656850). Afg3l1 possesses both ATPase and protease activities: the ATPase activity is required to unfold substrates, threading them into the internal proteolytic cavity for hydrolysis into small peptide fragments (By similarity). The m-AAA protease exerts a dual role in the mitochondrial inner membrane: it mediates the processing of specific regulatory proteins and ensures protein quality control by degrading misfolded polypeptides (By similarity). Required for SPG7 maturation into its active mature form after SPG7 cleavage by mitochondrial-processing peptidase (MPP) (PubMed:19656850). {ECO:0000250|UniProtKB:Q9Y4W6, ECO:0000269|PubMed:11549317, ECO:0000269|PubMed:19656850}.
uniprot
proteins
mass_dalton
87047
uniprot
proteins
mutagenesis
MUTAGEN 567; /note="E->Q: Absence of proteolytic activity. Loss of its processing into the mature form."; /evidence="ECO:0000269|PubMed:19656850
uniprot
proteins
protein_family
AAA ATPase family; Peptidase M41 family
uniprot
proteins
pubmed
11549317
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
17101804
uniprot
proteins
pubmed
19656850
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
27911893
uniprot
proteins
sequence_length
789
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000269|PubMed:11549317, ECO:0000269|PubMed:19656850}; Multi-pass membrane protein {ECO:0000255}.
uniprot
proteins
transmembrane_region
TRANSMEM 139..158; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 246..264; /note="Helical"; /evidence="ECO:0000255
uniprot
proteins