UniProt:P41247 PNPLA4

chain
  • chain:1-253
checksum 6FFA94F460BA03AC
comment
  • FUNCTION Has abundant triacylglycerol lipase activity (PubMed:15364929, PubMed:16150821, PubMed:17603008). Transfers fatty acid from triglyceride to retinol, hydrolyzes retinylesters, and generates 1,3-diacylglycerol from triglycerides (PubMed:17603008). Additionally possesses acylglycerol transacylase and phospholipase A2 activities (PubMed:15364929, PubMed:17603008).CATALYTIC ACTIVITY a triacylglycerol + H2O = a diacylglycerol + a fatty acid + H(+)CATALYTIC ACTIVITY a 1,2-diacyl-sn-glycero-3-phosphocholine + H2O = a 1-acyl-sn-glycero-3-phosphocholine + a fatty acid + H(+)CATALYTIC ACTIVITY an all-trans-retinyl ester + H2O = all-trans-retinol + a fatty acid + H(+)CATALYTIC ACTIVITY 2 a 1-acylglycerol = a 1,2-diacylglycerol + glycerolCATALYTIC ACTIVITY a 1-acylglycerol + a 1,2-diacylglycerol = a triacylglycerol + glycerolCATALYTIC ACTIVITY a 1-acylglycerol + a 1,3-diacylglycerol = a triacylglycerol + glycerolCATALYTIC ACTIVITY a triacylglycerol + H2O = a 1,2-diacylglycerol + a fatty acid + H(+)CATALYTIC ACTIVITY a triacylglycerol + H2O = a 1,3-diacylglycerol + a fatty acid + H(+)CATALYTIC ACTIVITY a triacylglycerol + all-trans-retinol = an all-trans-retinyl ester + a diacylglycerolCATALYTIC ACTIVITY 2 1-(9Z-octadecenoyl)-glycerol = 1,2-di-(9Z-octadecenoyl)-glycerol + glycerolCATALYTIC ACTIVITY 1-(9Z-octadecenoyl)-glycerol + 1,2-di-(9Z-octadecenoyl)-glycerol = 1,2,3-tri-(9Z-octadecenoyl)-glycerol + glycerolCATALYTIC ACTIVITY 1-(9Z-octadecenoyl)-glycerol + 1,3-di-(9Z-octadecenoyl)-glycerol = 1,2,3-tri-(9Z-octadecenoyl)-glycerol + glycerolCATALYTIC ACTIVITY 1,2-di-(9Z-octadecenoyl)-glycerol + (9Z)-octadecenoate + H(+) = 1,2,3-tri-(9Z-octadecenoyl)-glycerol + H2OCATALYTIC ACTIVITY 1,2,3-tri-(9Z-octadecenoyl)-glycerol + H2O = 1,3-di-(9Z-octadecenoyl)-glycerol + (9Z)-octadecenoate + H(+)CATALYTIC ACTIVITY all-trans-retinyl hexadecanoate + H2O = all-trans-retinol + hexadecanoate + H(+)CATALYTIC ACTIVITY 1,2,3-tri-(9Z-octadecenoyl)-glycerol + all-trans-retinol = all-trans-retinyl 9Z-octadecenoate + di-(9Z)-octadecenoylglycerolACTIVITY REGULATION The triglyceride lipase activity is inhibited by BEL ((E)-6-(bromomethylene)-3-(1-naphthalenyl)-2H-tetrahydropyran-2-one), a suicide substrate inhibitor.SUBCELLULAR LOCATION Expressed in all tissues examined, including heart, brain, placenta, lung, liver, muscle, kidney, pancreas and spleen.DISEASE Defects in PNPLA4 may play a role in mitochondrial disorders characterized by complex IV deficiency.
crossReference
databaseName UniProt
dbId 55984
description
  • recommendedName: Patatin-like phospholipase domain-containing protein 4 ecNumber evidence="2 4 5"3.1.1.3 alternativeName: fullName evidence="9"Calcium-independent phospholipase A2-eta shortName evidence="9"iPLA2-eta ecNumber evidence="2"3.1.1.4 alternativeName: fullName evidence="10"Protein GS2
displayName UniProt:P41247 PNPLA4
geneName
  • PNPLA4
  • DXS1283E
  • GS2
identifier P41247
isSequenceChanged false
keyword
  • Alternative splicing
  • Hydrolase
  • Lipid degradation
  • Lipid metabolism
  • Mitochondrion
  • Primary mitochondrial disease
  • Proteomics identification
  • Reference proteome
modified [InstanceEdit:9939033] Weiser, Joel, 2025-02-21
moleculeType Protein
name
  • PNPLA4
otherIdentifier
  • 11720862_at
  • 11720863_at
  • 11758008_s_at
  • 17108943
  • 209739_PM_s_at
  • 209739_s_at
  • 209740_PM_s_at
  • 209740_s_at
  • 35173_at
  • 3998641
  • 3998642
  • 3998643
  • 3998644
  • 3998645
  • 3998646
  • 3998647
  • 3998649
  • 3998651
  • 3998652
  • 3998653
  • 3998654
  • 3998655
  • 3998656
  • 3998657
  • 3998658
  • 8171229
  • 8228
  • A_23_P159797
  • A_24_P943815
  • GE573025
  • GO:0003824
  • GO:0004623
  • GO:0004806
  • GO:0005737
  • GO:0005739
  • GO:0005811
  • GO:0005829
  • GO:0006629
  • GO:0016020
  • GO:0016042
  • GO:0016411
  • GO:0016740
  • GO:0016787
  • GO:0019433
  • GO:0042572
  • GO:0043226
  • GO:0047376
  • GO:0050253
  • GO:0051264
  • GO:0051265
  • GO:0055088
  • HMNXSV003051319
  • Hs.264.0.S1_3p_a_at
  • ILMN_1664348
  • PH_hs_0000034
  • TC0X000827.hg
  • U03886_at
  • g458225_3p_a_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • PLPL4_HUMAN
  • A8K1H3
  • B4E362
  • Q8WW83
sequenceLength 253
species [Species:48887] Homo sapiens
stId uniprot:P41247
url http://purl.uniprot.org/uniprot/P41247
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