Spg7
entrez
353231
canonical
uniprot
Q7TT47
canonical
ensembl
ENSRNOG00000015150
resolver
ensembl
ENSRNOG00055021260
resolver
ensembl
ENSRNOG00060018191
resolver
ensembl
ENSRNOG00065018779
resolver
ensp
ENSRNOP00000048848
resolver
ensp
ENSRNOP00000073276
resolver
enst
ENSRNOT00000047754
resolver
genesymbol
Spg7
resolver
uniprot
A0A0G2K536
resolver
uniprot
A0A2H2GPM9
resolver
uniprot_entry
A0A2H2GPM9_RAT
resolver
uniprot_entry
SPG7_RAT
resolver
uniprot_sec
A0A0G2K536
resolver
amino_acid_sequence
MAAALLLLRALRQSPEPGPWRLWAQLSGRSPGLFSGAGGRRPYVVRGTPIGLAAAGGHTPQSLLLRILTPSFEGVSGLLLKRHIVPSAIRLWQLSGSTLYFNTSGLKQKNKDDDKPKGKAPEDDEEERRRKEREDQMYRERLRTLFIIAIVMSLLNSLSTSGGSISWADFVNEMLAKGEVQRVQVVPESDVVEVYLHPGAVVFGRPRLALMYRMQVANIDKFEEKLRAAEDELNIESKDRIPVSYKRTGFFGNALYALGMTAVGLAILWYVFRLAGMTGREGGFSAFNQLKMARFTIVDGKTGKGVSFQDVAGMHEAKLEVREFVDYLKSPERFLQLGAKVPKGALLLGPPGCGKTLLAKAVATEAQVPFLAMAGPEFVEVIGGLGAARVRSLFKEARARAPCIVYIDEIDAVGKKRSTSMSGFSNTEEEQTLNQLLVEMDGMGTADHVIVLASTNRADVLDNALMRPGRLDRHVFIDLPTLQERREIFEQHLKGLKLTQPSSFYSQRLAELTPGFSGADIANICNEAALHAAREGHTSVHTFNFEYAVERVIAGTAKKSKILSKEEQRVVAFHESGHALVGWLLEHTEAVMKVSIAPRTNAALGFSQMLPRDQYLFTKEQLFERMCMALGGRAAEAISFSRVTSGAQDDLRKVTRIAYSMVKQFGMAPSIGPVSFPEAQEGLVGIGRRPFSQGLQQMMDHEARLLVARAYRHTEKVLLDNLDKLQALANALLEKEVINYEDIEALIGPPPHGPKKMIAPQKWIDAEKEKQASGEEEAPAP
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 (By similarity). 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 (By similarity). 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:Q3ULF4, ECO:0000250|UniProtKB:Q9UQ90, ECO:0000250|UniProtKB:Q9Y4W6}.
uniprot
proteins
mass_dalton
86103
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 AFG3L2 removing an additional N-terminal fragment to generate the proteolytically active mature form. {ECO:0000250|UniProtKB:Q3ULF4}.
uniprot
proteins
protein_family
AAA ATPase family; Peptidase M41 family
uniprot
proteins
pubmed
15057822
uniprot
proteins
pubmed
26387735
uniprot
proteins
sequence_length
781
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000250|UniProtKB:Q9UQ90}; Multi-pass membrane protein {ECO:0000255}.
uniprot
proteins
transmembrane_region
TRANSMEM 145..165; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 249..269; /note="Helical"; /evidence="ECO:0000255
uniprot
proteins