UniProt:Q9H300 PARL

chain
  • transit peptide:1-52
  • chain:53-379
  • peptide:53-77
checksum 2B07EF04C7130B0B
comment
  • 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). Also required for cleavage of STARD7 and TTC19 (PubMed:28288130). Promotes changes in mitochondria morphology regulated by phosphorylation of P-beta domain (PubMed:14732705, PubMed:17116872).CATALYTIC ACTIVITY Cleaves type-1 transmembrane domains using a catalytic dyad composed of serine and histidine that are contributed by different transmembrane domains.SUBUNIT Interacts with PSEN1 and PSEN2. Binds OPA1.SUBCELLULAR LOCATION Translocated into the nucleus by an unknown mechanism (PubMed:17116872).ALTERNATIVE PRODUCTS 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.SIMILARITY Belongs to the peptidase S54 family.
created [InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
crossReference
databaseName UniProt
dbId 241699
description
  • recommendedName: Presenilin-associated rhomboid-like protein, mitochondrial ecNumber evidence="7"3.4.21.105 alternativeName: Mitochondrial intramembrane cleaving protease PARL component recommendedName: P-beta shortName: Pbeta /component
displayName UniProt:Q9H300 PARL
geneName
  • PARL
  • PSARL
  • PRO2207
identifier Q9H300
isSequenceChanged false
keyword
  • Alternative splicing
  • Direct protein sequencing
  • Hydrolase
  • Membrane
  • Mitochondrion
  • Mitochondrion inner membrane
  • Nucleus
  • Phosphoprotein
  • Protease
  • Proteomics identification
  • Reference proteome
  • Serine protease
  • Transit peptide
  • Transmembrane
  • Transmembrane helix
modified [InstanceEdit:9926675] Weiser, Joel, 2024-11-03
moleculeType Protein
name
  • PARL
otherIdentifier
  • 11731166_a_at
  • 16962112
  • 218271_PM_s_at
  • 218271_s_at
  • 2708231
  • 2708232
  • 2708233
  • 2708234
  • 2708236
  • 2708237
  • 2708238
  • 2708240
  • 2708244
  • 2708245
  • 2708246
  • 2708247
  • 2708252
  • 2708253
  • 2708256
  • 2708258
  • 2708264
  • 2708265
  • 55486
  • 57809_at
  • 8092409
  • A_24_P221883
  • A_33_P3335451
  • GE82427
  • GE87045
  • GO:0004175
  • GO:0004252
  • GO:0005515
  • GO:0005634
  • GO:0005739
  • GO:0005743
  • GO:0006465
  • GO:0006508
  • GO:0007005
  • GO:0008053
  • GO:0008233
  • GO:0008236
  • GO:0010821
  • GO:0016020
  • GO:0016485
  • GO:0016787
  • GO:0030162
  • GO:0033619
  • GO:0090201
  • GO:1901524
  • GO:1903214
  • GO:2000377
  • GO:2001243
  • HMNXSV003004035
  • HMNXSV003044582
  • ILMN_1731354
  • ILMN_2257665
  • ILMN_2341467
  • PH_hs_0001259
  • PH_hs_0031004
  • TC03002062.hg
  • g8924133_3p_s_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • PARL_HUMAN
  • Q96CQ4
  • Q9BTJ6
  • Q9P1E3
sequenceLength 379
species [Species:48887] Homo sapiens
stId uniprot:Q9H300
url http://purl.uniprot.org/uniprot/Q9H300
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