PARL
entrez
55486
canonical
uniprot
Q9H300
canonical
ensembl
ENSG00000175193
resolver
ensp
ENSP00000310676
resolver
ensp
ENSP00000325421
resolver
ensp
ENSP00000402137
resolver
ensp
ENSP00000644220
resolver
enst
ENST00000435888
resolver
genesymbol
PARL
resolver
genesymbol_syn
PSARL
resolver
hgnc
HGNC:18253
resolver
uniprot
C9JNP8
resolver
uniprot
Q96CQ4
resolver
uniprot
Q9BTJ6
resolver
uniprot
Q9P1E3
resolver
uniprot_entry
C9JNP8_HUMAN
resolver
uniprot_entry
PARL_HUMAN
resolver
uniprot_sec
Q96CQ4
resolver
uniprot_sec
Q9BTJ6
resolver
uniprot_sec
Q9P1E3
resolver
amino_acid_sequence
MAWRGWAQRGWGCGQAWGASVGGRSCEELTAVLTPPQLLGRRFNFFIQQKCGFRKAPRKVEPRRSDPGTSGEAYKRSALIPPVEETVFYPSPYPIRSLIKPLFFTVGFTGCAFGSAAIWQYESLKSRVQSYFDGIKADWLDSIRPQKEGDFRKEINKWWNNLSDGQRTVTGIIAANVLVFCLWRVPSLQRTMIRYFTSNPASKVLCSPMLLSTFSHFSLFHMAANMYVLWSFSSSIVNILGQEQFMAVYLSAGVISNFVSYVGKVATGRYGPSLGASGAIMTVLAAVCTKIPEGRLAIIFLPMFTFTAGNALKAIIAMDTAGMILGWKFFDHAAHLGGALFGIWYVTYGHELIWKNREPLVKIWHEIRTNGPKKGGGSK
uniprot
proteins
ec_number
3.4.21.105
uniprot
proteins
function
FUNCTION: Required for the control of apoptosis during postnatal growth. Essential for proteolytic processing of an antiapoptotic form of OPA1 which prevents the release of mitochondrial cytochrome c in response to intrinsic apoptotic signals (By similarity). Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP) (PubMed:22354088). Promotes cleavage of serine/threonine-protein phosphatase PGAM5 in damaged mitochondria in response to loss of mitochondrial membrane potential (PubMed:22915595). Mediates differential cleavage of PINK1 and PGAM5 depending on the health status of mitochondria, disassociating from PINK1 and associating with PGAM5 in response to mitochondrial membrane potential loss (PubMed:22915595). Required for processing of CLPB into a form with higher protein disaggregase activity by removing an autoinhibitory N-terminal peptide (PubMed:28288130, PubMed:32573439). Promotes processing of DIABLO/SMAC in the mitochondrion which is required for DIABLO apoptotic activity (PubMed:28288130). Mediates STARD7 processing which regulates STARD7 location between mitochondria and cytosol (PubMed:28288130, PubMed:29301859, PubMed:36658222). Also required for cleavage of TTC19 (PubMed:28288130). Promotes changes in mitochondria morphology regulated by phosphorylation of P-beta domain (PubMed:14732705, PubMed:17116872). {ECO:0000250|UniProtKB:Q5XJY4, ECO:0000269|PubMed:14732705, ECO:0000269|PubMed:17116872, ECO:0000269|PubMed:22354088, ECO:0000269|PubMed:22915595, ECO:0000269|PubMed:28288130, ECO:0000269|PubMed:29301859, ECO:0000269|PubMed:32573439, ECO:0000269|PubMed:36658222}.
uniprot
proteins
mass_dalton
42190
uniprot
proteins
mutagenesis
MUTAGEN 65; /note="S->D: Strongly reduces the beta cleavage; when associated with D-69 and D-70."; /evidence="ECO:0000269|PubMed:17116872"; MUTAGEN 69; /note="T->D: Strongly reduces the beta cleavage; when associated with D-65 and D-70."; /evidence="ECO:0000269|PubMed:17116872"; MUTAGEN 70; /note="S->D: Strongly reduces the beta cleavage; when associated with D-65 and D-69."; /evidence="ECO:0000269|PubMed:17116872"; MUTAGEN 76; /note="R->E: Abolishes the beta cleavage."; /evidence="ECO:0000269|PubMed:14732705"; MUTAGEN 76; /note="R->G: Abolishes the beta cleavage."; /evidence="ECO:0000269|PubMed:14732705"; MUTAGEN 77; /note="S->E: Abolishes the beta cleavage."; /evidence="ECO:0000269|PubMed:14732705"; MUTAGEN 78; /note="A->E: Abolishes the beta cleavage."; /evidence="ECO:0000269|PubMed:14732705"; MUTAGEN 79; /note="L->E: Abolishes the beta cleavage."; /evidence="ECO:0000269|PubMed:14732705"; MUTAGEN 277; /note="S->A: Loss of cleavage of CLPB, DIABLO, STARD7 and TTC19."; /evidence="ECO:0000269|PubMed:28288130, ECO:0000269|PubMed:29301859"; MUTAGEN 277; /note="S->G: Loss of PGAM5 cleavage."; /evidence="ECO:0000269|PubMed:22915595"; MUTAGEN 335; /note="H->A: Loss of PGAM5 cleavage."; /evidence="ECO:0000269|PubMed:22915595
uniprot
proteins
post_translational_modification
PTM: P-beta is proteolytically processed (beta-cleavage) in a PARL-dependent manner. The cleavage is inhibited when residues Ser-65, Thr-69 and Ser-70 are all phosphorylated. {ECO:0000269|PubMed:17116872}.
uniprot
proteins
protein_family
Peptidase S54 family
uniprot
proteins
pubmed
12214059
uniprot
proteins
pubmed
14732705
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
17116872
uniprot
proteins
pubmed
22354088
uniprot
proteins
pubmed
22915595
uniprot
proteins
pubmed
28288130
uniprot
proteins
pubmed
29301859
uniprot
proteins
pubmed
32573439
uniprot
proteins
pubmed
36658222
uniprot
proteins
sequence_length
379
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000269|PubMed:17116872, ECO:0000269|PubMed:22354088, ECO:0000269|PubMed:29301859}; Multi-pass membrane protein {ECO:0000269|PubMed:17116872}.; SUBCELLULAR LOCATION: [P-beta]: Nucleus {ECO:0000269|PubMed:17116872}. Note=Translocated into the nucleus by an unknown mechanism (PubMed:17116872). {ECO:0000269|PubMed:17116872}.
uniprot
proteins
transmembrane_region
TRANSMEM 102..121; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 168..187; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 208..230; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 245..262; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 273..289; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 296..318; /note="Helical"; /evidence="ECO:0000255"; TRANSMEM 333..354; /note="Helical"; /evidence="ECO:0000255
uniprot
proteins