PNPLA4
entrez
8228
canonical
uniprot
P41247
canonical
ensembl
ENSG00000006757
resolver
ensp
ENSP00000370430
resolver
ensp
ENSP00000415245
resolver
ensp
ENSP00000443157
resolver
genesymbol
PNPLA4
resolver
genesymbol_syn
DXS1283E
resolver
genesymbol_syn
GS2
resolver
hgnc
HGNC:24887
resolver
uniprot
A8K1H3
resolver
uniprot
B4E362
resolver
uniprot
Q8WW83
resolver
uniprot_entry
PLPL4_HUMAN
resolver
uniprot_sec
A8K1H3
resolver
uniprot_sec
B4E362
resolver
uniprot_sec
Q8WW83
resolver
activity_regulation
ACTIVITY REGULATION: The triglyceride lipase activity is inhibited by BEL ((E)-6-(bromomethylene)-3-(1-naphthalenyl)-2H-tetrahydropyran-2-one), a suicide substrate inhibitor. {ECO:0000269|PubMed:15364929}.
uniprot
proteins
amino_acid_sequence
MKHINLSFAACGFLGIYHLGAASALCRHGKKLVKDVKAFAGASAGSLVASVLLTAPEKIEECNQFTYKFAEEIRRQSFGAVTPGYDFMARLRSGMESILPPSAHELAQNRLHVSITNAKTRENHLVSTFSSREDLIKVLLASSFVPIYAGLKLVEYKGQKWVDGGLTNALPILPVGRTVTISPFSGRLDISPQDKGQLDLYVNIAKQDIMLSLANLVRLNQALFPPSKRKMESLYQCGFDDTVKFLLKENWFE
uniprot
proteins
disease_involvement
DISEASE: Note=Defects in PNPLA4 may play a role in mitochondrial disorders characterized by complex IV deficiency. {ECO:0000269|PubMed:26741492}.
uniprot
proteins
ec_number
3.1.1.3
uniprot
proteins
ec_number
3.1.1.4
uniprot
proteins
function
FUNCTION: Has abundant triacylglycerol lipase activity (PubMed:15364929, PubMed:15955102, PubMed:16150821, PubMed:17603008). Transfers fatty acid from triglyceride to retinol, hydrolyzes retinylesters, and generates 1,3-diacylglycerol from triglycerides (PubMed:15955102, PubMed:17603008). Additionally possesses acylglycerol transacylase and phospholipase A2 activities (PubMed:15364929, PubMed:17603008). Able to catalyze fatty acyl CoA-dependent retinol esterification during epidermal maturation, when pH becomes acidic (PubMed:15955102). {ECO:0000269|PubMed:15364929, ECO:0000269|PubMed:15955102, ECO:0000269|PubMed:16150821, ECO:0000269|PubMed:17603008}.
uniprot
proteins
mass_dalton
27980
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15364929
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15772651
uniprot
proteins
pubmed
15955102
uniprot
proteins
pubmed
16150821
uniprot
proteins
pubmed
17603008
uniprot
proteins
pubmed
26741492
uniprot
proteins
pubmed
7806223
uniprot
proteins
sequence_length
253
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion {ECO:0000269|PubMed:26741492}.
uniprot
proteins