Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

3.1.1.34

Official Name

lipoprotein lipase

Name from literature

Lipoprotein lipase

Pathway from literature

the hydrolysis of triglyceride-rich lipoproteins and plays an important role in glucose metabolism

Pathway from KEGG

Lipid Metabolism; Glycerolipid metabolism; map00561

Human Diseases; Neurodegenerative Diseases; Alzheimer's disease; map05010

Cellular Processes; Endocrine System; PPAR signaling pathway; map03320

Organisms

Human (9606)

Genome localization

8p22[4023 ],

Comments

Hydrolyses triacylglycerols in chylomicrons and low-density lipoproteins. Also hydrolyses diacylglycerol.

Rate-limiting Description

"Lipoprotein lipase (LPL) is the rate-limiting enzyme in the hydrolysis of serum triglycerides associated with the lipoprotein particles very low density lipoprotein and chylomicrons." (10650951)

"The tissue-specific expression of lipoprotein lipase (LPL) in adipose tissue (AT), skeletal muscle (SM), and cardiac muscle (CM) is rate-limiting for the uptake of triglyceride (TG)-derived free fatty acids and decisive in the regulation of energy balance and lipoprotein metabolism." (10077655)

Regulatory Information

Upstream transcription factor

6667;6670

Regulatory type

Detail

transcriptional factor;"Sp1(6667),Sp3(6670)"

"role of Sp1 and Sp3 in interferon-gamma mediated suppression of gene transcription" (11796707)

Gene ontology

Gene ontology

GO:0031225 (C) anchored to membrane [P06858 ];
GO:0006644 (P) phospholipid metabolic process [P06858 ];
GO:0008015 (P) blood circulation [P06858 ];
GO:0006641 (P) triacylglycerol metabolic process [P06858 ];
GO:0005886 (C) plasma membrane [P06858 ];
GO:0004465 (F) lipoprotein lipase activity [P06858 ];
GO:0005102 (F) receptor binding [P06858 ];
GO:0042627 (C) chylomicron [P06858 ];
GO:0005319 (F) lipid transporter activity [P06858 ];
GO:0004806 (F) triacylglycerol lipase activity [P06858 ];
GO:0004620 (F) phospholipase activity [P06858 ];
GO:0016042 (P) lipid catabolic process [P06858 ];
GO:0008201 (F) heparin binding [P06858 ];

Subcellular localization

Localization

cell membrane [P06858 ];

Disease relevance

Disease

Defects in LPL are a cause of familial chylomicronemia [MIM:238600];
also known as hyperlipoproteinemia type I. 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 [P06858 ];

Defects in LPL are the cause of lipoprotein lipase deficiency (LPL deficiency) [MIM:238600]. LPL deficiency leads to hypertriglyceridemia [P06858 ];

Links

SwissProt

P06858

Entrez Gene

4023

HPRD

01999



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  Last Modified: 2009-03-24  
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