Names & Taxonomy

Uniprot ID:
Q9Y4W6
Entry Name:
AFG32_HUMAN
Status:
reviewed
Protein Names:
AFG3-like protein 2 (EC 3.4.24.-) (Paraplegin-like protein)
Gene Names:
AFG3L2
Gene Names Primary:
AFG3L2
Organism:
Homo sapiens (Human)

Structure

Length:
797
Sequence:
MAHRCLRLWGRGGCWPRGLQQLLVPGGVGPGEQPCLRTLYRFVTTQARASRNSLLTDIIAAYQRFCSRPPKGFEKYFPNGKNGKKASEPKEVMGEKKESKPAATTRSSGGGGGGGGKRGGKKDDSHWWSRFQKGDIPWDDKDFRMFFLWTALFWGGVMFYLLLKRSGREITWKDFVNNYLSKGVVDRLEVVNKRFVRVTFTPGKTPVDGQYVWFNIGSVDTFERNLETLQQELGIEGENRVPVVYIAESDGSFLLSMLPTVLIIAFLLYTIRRGPAGIGRTGRGMGGLFSVGETTAKVLKDEIDVKFKDVAGCEEAKLEIMEFVNFLKNPKQYQDLGAKIPKGAILTGPPGTGKTLLAKATAGEANVPFITVSGSEFLEMFVGVGPARVRDLFALARKNAPCILFIDEIDAVGRKRGRGNFGGQSEQENTLNQLLVEMDGFNTTTNVVILAGTNRPDILDPALLRPGRFDRQIFIGPPDIKGRASIFKVHLRPLKLDSTLEKDKLARKLASLTPGFSGADVANVCNEAALIAARHLSDSINQKHFEQAIERVIGGLEKKTQVLQPEEKKTVAYHEAGHAVAGWYLEHADPLLKVSIIPRGKGLGYAQYLPKEQYLYTKEQLLDRMCMTLGGRVSEEIFFGRITTGAQDDLRKVTQSAYAQIVQFGMNEKVGQISFDLPRQGDMVLEKPYSEATARLIDDEVRILINDAYKRTVALLTEKKADVEKVALLLLEKEVLDKNDMVELLGPRPFAEKSTYEEFVEGTGSLDEDTSLPEGLKDWNKEREKEKEEPPGEKVAN
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion membrane

Function

Function:
ATP-dependent protease which is essential for axonal development.
Cofactor:
COFACTOR: Name=Zn(2+); Xref=ChEBI:CHEBI:29105;
Active Site:
ACT_SITE 575 575
Cross Reference Drug Bank:
DB00171
Gene Ontology Go:
integral component of membrane
mitochondrial inner membrane
mitochondrion
ATP binding
ATP-dependent peptidase activity
metalloendopeptidase activity
metallopeptidase activity
unfolded protein binding
zinc ion binding
axonogenesis
cristae formation
mitochondrial fusion
mitochondrial protein processing
muscle fiber development
myelination
nerve development
neuromuscular junction development
protein complex assembly
protein import into mitochondrial intermembrane space
regulation of multicellular organism growth
righting reflex
Gene Ontology Biological Process:
axonogenesis
cristae formation
mitochondrial fusion
mitochondrial protein processing
muscle fiber development
myelination
nerve development
neuromuscular junction development
protein complex assembly
protein import into mitochondrial intermembrane space
regulation of multicellular organism growth
righting reflex
Gene Ontology Molecular Function:
ATP binding
ATP-dependent peptidase activity
metalloendopeptidase activity
metallopeptidase activity
unfolded protein binding
zinc ion binding
Gene Ontology Cellular Component:
integral component of membrane
mitochondrial inner membrane
mitochondrion
Keywords:
3D-structure
ATP-binding
Complete proteome
Disease mutation
Hydrolase
Membrane
Metal-binding
Metalloprotease
Mitochondrion
Neurodegeneration
Nucleotide-binding
Protease
Reference proteome
Spinocerebellar ataxia
Transmembrane
Transmembrane helix
Zinc

Publication

PubMed ID:
10395799 15489334 14623864 17101804 21269460 24275569 25944712 20354562 20725928 20208537 22022284 24293060 26677414