Lipoprotein lipase (IPR002330)

Short name: Lipo_Lipase

Overlapping homologous superfamilies

Family relationships


Lipoprotein lipase (LPL) is a key enzyme of lipid metabolism that hydrolyses triglycerides, providing free fatty acids for cells and affecting the maturation of circulating lipoproteins [PMID: 3823907]. The enzyme is thought to play a role in the development of obesity and atherosclerosis [PMID: 3823907]. Human LPL contains 448 amino acids; sequence comparison indicates that human LPL, hepatic lipase, and pancreatic lipase are members of a gene family [PMID: 3823907]. Defects in LPL are a cause of familial chylomicronemia syndrome (or type I hyperlipoproteinemia) and also of a form of deficiency characterised by hypertriglyceridemia. Familial chylomicronemia is a recessive disorder usually manifesting in childhood. On a normal diet, patients often present with abdominal pain, hepatosplenomegaly, lipemia retinalis, eruptive xanthomata, and massive hypertriglyceridemia, sometimes complicated with acute pancreatitis. LPL and pancreatic lipase share ~30% sequence identity, suggesting a similar tertiary fold [PMID: 8308035]. Molecular models of LPL have been constructed, based on X-ray crystal structures of pancreatic lipase [PMID: 1479292]. These models allowed the authors to propose hypotheses on the structural determinants of LPL that are responsible for heparin binding, dimer formation, and phospholipase activity.

GO terms

Biological Process

GO:0006629 lipid metabolic process

Molecular Function

GO:0004465 lipoprotein lipase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.