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Details on Person UniProt:P41247 PNPLA4

Class:Id	ReferenceGeneProduct:55984
_chainChangeLog	chain:1-253 added on Fri February 6 2015
_displayName	UniProt:P41247 PNPLA4
_timestamp	2025-02-21 19:00:18
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.
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
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:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9909836] Weiser, Joel, 2024-05-14 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21
name	PNPLA4
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:8994430] ENSEMBL:ENSG00000006757 PNPLA4 [Homo sapiens]
secondaryIdentifier	PLPL4_HUMAN A8K1H3 B4E362 Q8WW83
sequenceLength	253
species	[Species:48887] Homo sapiens
(isoformParent)	[ReferenceIsoform:8977147] UniProt:P41247-1 PNPLA4 [Homo sapiens] [ReferenceIsoform:8977148] UniProt:P41247-2 PNPLA4 [Homo sapiens]
(referenceEntity)	[EntityWithAccessionedSequence:8848356] PNPLA4 [cytosol] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:P41247 PNPLA4 (55984)