Spg7
entrez
234847
canonical
uniprot
Q3ULF4
canonical
ensembl
ENSMUSG00000000738
resolver
ensp
ENSMUSP00000119552
resolver
ensp
ENSMUSP00000231537
resolver
enst
ENSMUST00000149248
resolver
genesymbol
Spg7
resolver
uniprot
B2RQY8
resolver
uniprot
D3Z1Z1
resolver
uniprot
Q4V9T9
resolver
uniprot
Q7TNG0
resolver
uniprot
Q80X42
resolver
uniprot
Q811Y5
resolver
uniprot
Q8K414
resolver
uniprot
Q8R1A1
resolver
uniprot
Q8R1K2
resolver
uniprot_entry
SPG7_MOUSE
resolver
uniprot_sec
B2RQY8
resolver
uniprot_sec
D3Z1Z1
resolver
uniprot_sec
Q4V9T9
resolver
uniprot_sec
Q7TNG0
resolver
uniprot_sec
Q80X42
resolver
uniprot_sec
Q811Y5
resolver
uniprot_sec
Q8K414
resolver
uniprot_sec
Q8R1A1
resolver
uniprot_sec
Q8R1K2
resolver
amino_acid_sequence
MAAALLLLRGLRPGPEPRPRRLWGLLSGRGPGLSSGAGARRPYAARGTPVGPAAAGGHAPQSLLLRILTPSFEGISGLLLKQHIVPNAVRLWPLSGSTLYFNTSRMKQKNKDNDKPKGKTPEDDEEEKRRKEREDQMYRERLRTLFIIALVMSLLNSLSTSGGSISWADFVNEMLAKGEVQRVQVVPESDVVEVYLHPGAVVFGRPRLALMYRMQVANIDKFEEKLRAAEDELNIESKDRIPVSYKRTGFFGNALYALGMTAVGLAILWYVFRLAGMTGREGGFSAFNQLKMARFTIVDGKTGKGVSFQDVAGMHEAKLEVREFVDYLKSPERFLQLGAKVPKGALLLGPPGCGKTLLAKAVATEAQVPFLAMAGPEFVEVIGGLGAARVRSLFKEARARAPCIVYIDEIDAVGKKRSTSMSGFSNTEEEQTLNQLLVEMDGMGTTDHVIVLASTNRADVLDNALMRPGRLDRHVFIDLPTLQERREIFEQHLKGLKLTQPSSFYSQRLAELTPGFSGADIANICNEAALHAAREGHTSVHTFNFEYAVERVIAGTAKKSKILSKEEQRVVAFHESGHALVGWLLEHTEAVMKVSIAPRTNAALGFSQMLPRDQYLFTKEQLFERMCMALGGRAAEAISFSRVTSGAQDDLRKVTRIAYSMVKQFGMAPSIGPVSFPEAQEGLMGIGRRPFSQGLQQMMDHEAKLLVAKAYRHTEKVLLDNLDKLQALANALLEKEVINYEDIEALIGPPPHGPKKMIAPQKWIDAEKERQASGEEEAPAP
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:16239145). SPG7 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). Mediates protein maturation of the mitochondrial ribosomal subunit MRPL32/bL32m by catalyzing the cleavage of the presequence of MRPL32/bL32m prior to assembly into the mitochondrial ribosome (PubMed:16239145). Acts as a regulator of calcium in neurons by mediating degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU (By similarity). Also regulates mitochondrial calcium by catalyzing degradation of MCU (By similarity). Plays a role in the formation and regulation of the mitochondrial permeability transition pore (mPTP) and its proteolytic activity is dispensable for this function (By similarity). {ECO:0000250|UniProtKB:Q9UQ90, ECO:0000250|UniProtKB:Q9Y4W6, ECO:0000269|PubMed:16239145}.
uniprot
proteins
mass_dalton
85996
uniprot
proteins
mutagenesis
MUTAGEN 575; /note="E->Q: Absence of proteolytic activity. No loss of its processing into the mature form."; /evidence="ECO:0000269|PubMed:19656850
uniprot
proteins
post_translational_modification
PTM: Upon import into the mitochondrion, the N-terminal transit peptide is cleaved by the mitochondrial-processing peptidase (MPP) to generate an intermediate form which undergoes a second proteolytic cleavage mediated by proteases AFG3L1 and/or AFG3L2 removing an additional N-terminal fragment to generate the proteolytically active mature form. {ECO:0000269|PubMed:19656850}.
uniprot
proteins
protein_family
AAA ATPase family; Peptidase M41 family
uniprot
proteins
pubmed
14722615
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
16239145
uniprot
proteins
pubmed
16800626
uniprot
proteins
pubmed
17101804
uniprot
proteins
pubmed
19468303
uniprot
proteins
pubmed
19656850
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
22563492
uniprot
proteins
sequence_length
781
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000269|PubMed:19656850, ECO:0000269|PubMed:22563492}; Multi-pass membrane protein {ECO:0000255}.
uniprot
proteins
transmembrane_region
TRANSMEM 145..165; /note="Helical; Name=1"; /evidence="ECO:0000255"; TRANSMEM 249..269; /note="Helical; Name=2"; /evidence="ECO:0000255
uniprot
proteins