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lipoprotein lipase (lipase-D, post heparin lipase, LPL, LIPD)
Function:
- hydrolysis of triglycerides of circulating chylomicrons & very low density lipoproteins (VLDL)
- functions in the presence of apoC2 on the luminal surface of vascular endothelium
- activity is dependent upon apoC2 acquired by chylomicrons or VLDL from HDL
- promotes production of chylomicron remnants from chylomicrons
- preferred substrate is triglyceride within chylomicrons.
- non covalently attached to surface of endothelial cells via membrane-bound heparan sulfate proteoglycan
- release into plasma following heparin administration is known as post-heparin lipolytic activity
- hepatic triglyceride lipase also contributes to post-heparin lipolytic activity
triacylglycerol + H2O diacylglycerol + a carboxylate
Structure:
- homodimer.
- interacts with apoC2
- belongs to the AB hydrolase superfamily, lipase family
- contains 1 PLAT domain
Compartment: cell membrane, heparan sulfate proteoglycan anchor
Pathology:
- deficiency in lipoprotein lipase leads to Federickson & Levy type 1 hyperlipoproteinemia (familial chylomicronemia)
- defects in LPL are a cause of type 5 hyperlipoproteinemia (Snomed)
Related
hyperlipoproteinemia type 1; familial hyperchylomicronemia
lipoprotein lipase deficiency
post-heparin lipolytic activity
General
glycoprotein
lipase
oligomerizing protein
plasma membrane protein
Properties
SIZE: entity length = 475 aa
MW = 53 kD
COMPARTMENT: plasma membrane
CELL-REGION: apical region
CELL: endothelial cell
MOTIF: signal sequence {1-27}
cysteine residue {C54}
MODIFICATION: cysteine residue {C67}
cysteine residue {C67}
MODIFICATION: cysteine residue {C54}
N-glycosylation site {N70}
serine residue {S159}
aspartate residue {D183}
cysteine residue {C243}
MODIFICATION: cysteine residue {C266}
cysteine residue {C266}
MODIFICATION: cysteine residue {C243}
histidine residue {H268}
cysteine residue {C291}
MODIFICATION: cysteine residue {C310}
cysteine residue {C302}
MODIFICATION: cysteine residue {C305}
cysteine residue {C305}
MODIFICATION: cysteine residue {C302}
cysteine residue {C310}
MODIFICATION: cysteine residue {C291}
binding site
SITE: 319-331
EFFECTOR-BOUND: heparan sulfate proteoglycan
PLAT {341-464}
MOTIF: N-glycosylation site {N386}
cysteine residue {C445}
MODIFICATION: cysteine residue {C465}
cysteine residue {C465}
MODIFICATION: cysteine residue {C445}
Database Correlations
OMIM 238600
UniProt P06858
PFAM correlations
KEGG correlations
ENZYME 3.1.1.34
References
- Primary Hyperlipoproteinemias, Steiner & Shafrir
(eds), McGraw Hill, NY, 1991, pg 28
- Andrews BS.
Is the WKS motif the tissue-factor binding site for
coagulation factor VII?
Trends Biochem Sci. 1991 Jan;16(1):31-6. Review.
PMID: 2053135
- Brouwer CB et al
Different clearance of intravenously administered olive oil
and soybean-oil emulsions: role of hepatic lipase.
Am J Clin Nutr 57:533 1993
PMID: 8460608
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=LP
- SHMPD, The Singapore human mutation and polymorphism database
http://shmpd.bii.a-star.edu.sg/gene.php?genestart=A&genename=LPL
- Wikipedia, lipoprotein lipase entry
http://en.wikipedia.org/wiki/lipoprotein_lipase
- UniProt :accession P06858