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

Basic Information

Enzyme Number

3.1.1.3

Official Name

triacylglycerol lipase

Name from literature

carboxyl ester lipase

Pathway from literature

severe pancreatic exocrine dysfunction

Pathway from KEGG

Lipid Metabolism; Glycerolipid metabolism; map00561

Organisms

Human (9606)

Genome localization

18q21.1[9388 ], 10q25.3[5407 ], 22q13.31[80339 ], 9q34.3[1056 ], 15q21-q23[3990 ], 10q26.1[5406 ], 10q25.3[5408 ], 10q23.31[8513 ], 10q23.2-q23.3[3988 ],

Comments

The pancreatic enzyme acts only on an ester-water interface; the outer ester links are preferentially hydrolysed.

Rate-limiting Description

"14CO2 was expired more rapidly than 13CO2, suggesting that hydrolysis of the substrate may also be rate limiting in healthy volunteers." (3678753)

Regulatory Information

Upstream transcription factor

10891;3172;6720;7026;6927

Regulatory type

Detail

transcriptional factor;SREBP-1(6720)

"the opposite regulation of HL expression by fatty acids and statins is mediated via SREBP, possibly through interaction with upstream stimulatory factors" (15721010)

transcriptional factor;"HNF4alpha(3172),HNF1alpha(6927),PGC-1alpha(10891),ARP-1(7026)"

"LIPC promoter is regulated by hepatocyte nuclear factor 4alpha(HNF4alpha), peroxisome proliferator-activated receptor gamma coactivator-1alpha(PGC-1alpha), apolipoprotein A-I regulatory protein-1(ARP-1), and hepatocyte nuclear factor 1alpha(HNF1alpha)" (16603721)

phosphorylation;

P11150

phosphorylation;

P41247

phosphorylation;

Q96AD5

Gene ontology

Gene ontology

GO:0006707 (P) cholesterol catabolic process [P19835 ];
GO:0019432 (P) triacylglycerol biosynthetic process [Q9NST1 ];
GO:0019433 (P) triacylglycerol catabolic process [Q9NST1 ];
GO:0034430 (C) monolayer-surrounded lipid storage body out... [Q96AD5 ];
GO:0005737 (C) cytoplasm [P41247, P19835 ];
GO:0008970 (F) phospholipase A1 activity [Q9Y5X9 ];
GO:0006487 (P) protein amino acid N-linked glycosylation [P11150 ];
GO:0005319 (F) lipid transporter activity [P11150 ];
GO:0016021 (C) integral to membrane [Q9NST1, Q96AD5 ];
GO:0008289 (F) lipid binding [P07098 ];
GO:0044258 (P) intestinal lipid catabolic process [P19835 ];
GO:0051265 (F) diolein transacylation activity [Q9NST1 ];
GO:0005515 (F) protein binding [Q9Y5X9 ];
GO:0030299 (P) cholesterol absorption [P19835 ];
GO:0051264 (F) mono-olein transacylation activity [Q9NST1 ];
GO:0030157 (P) pancreatic juice secretion [P19835 ];
GO:0004771 (F) sterol esterase activity [P19835 ];
GO:0009062 (P) fatty acid catabolic process [P19835 ];
GO:0004465 (F) lipoprotein lipase activity [Q9Y5X9 ];
GO:0005886 (C) plasma membrane [Q96AD5 ];
GO:0006641 (P) triacylglycerol metabolic process [P07098, P54317, P19835 ];
GO:0004623 (F) phospholipase A2 activity [Q9NST1 ];
GO:0047372 (F) acylglycerol lipase activity [P54317, P19835 ];
GO:0018350 (P) protein amino acid esterification [P19835 ];
GO:0005576 (C) extracellular region [P54315, Q17RR3, P07098, Q9Y5X9, P16233, P54317, P19835 ];
GO:0004806 (F) triacylglycerol lipase activity [P54315, Q17RR3, P07098, Q96AD5, P16233, P54317, P41247, Q9NST1, Q9Y5X9, P11150, P19835 ];
GO:0005634 (C) nucleus [P41247, P19835 ];
GO:0008201 (F) heparin binding [Q9Y5X9, P11150, P19835 ];
GO:0016042 (P) lipid catabolic process [P54315, Q17RR3, P07098, Q96AD5, P16233, P54317, P41247, Q9Y5X9, P11150 ];

Tissue expression

Tissue From HPRD

Thymus [04730 ];
Colon [04730 ];
Uterus [04730 ];
Chief cell [09063 ];
Corpus luteum [04730 ];
Testis [04730 ];
Macrophage [07509, 04730 ];
Hepatocyte [04730 ];
Skeletal muscle [02112, 04730 ];
Liver [02044, 02112, 04730, 01058 ];
Muscle [04730 ];
Mammary epithelium [04730 ];
Keratinocyte [04730 ];
Brain [02044, 02112, 04730 ];
Spleen [02112, 04730 ];
Prostate [02112 ];
Small intestine [04730 ];
Adipose tissue [12430 ];
Kidney [02044, 02112, 04730 ];
Smooth muscle [04730 ];
Mammary gland [02044, 07509 ];
Monocyte [07509, 04730 ];
Lung [02044, 02112, 04730 ];
Heart [02044, 02112, 04730 ];
Ovary [04730 ];
Pancreas [05109, 02112, 07509, 04730, 05110, 02005 ];
Placenta [02044, 02112, 04730 ];
Adrenal cortex [04730 ];
Adrenal medulla [04730 ];
Thyroid gland [04730 ];
Coronary artery [04730 ];
Stomach [04730, 09063 ];

Tissue specificity

Mammary gland and pancreas [P19835 ];

Pancreas [P54315, P54317 ];

High level of expression in the liver, placenta, lung, thyroid, kidney, testis and in the corpus luteum of the ovary. Expressed also in coronary artery endothelial cells, umbilical vein endothelial cells and in hepatocyte and osteosarcoma cell lines. Not detected in heart, brain and muscle [Q9Y5X9 ];

Highest expression in adipose tissue. Also detected in heart, skeletal muscle, and portions of the gastrointestinal tract. Detected in normal retina and retinoblastoma cells. Detected in retinal pigment epithelium and, at lower intensity, in the inner segments of photoreceptors and in the ganglion cell layer of the neural retina (at protein level) [Q96AD5 ];

Expressed in all tissues examined, including heart, brain, placenta, lung, liver, muscle, kidney, pancreas and spleen [P41247 ];

Subcellular localization

Localization

cell membrane [Q96AD5 ];

extracellular [P54315, Q17RR3, P07098, Q9Y5X9, P16233, P11150, P54317 ];

membrane [Q9NST1 ];

Disease relevance

Disease

Defects in LIPC are the cause of hepatic lipase deficiency (HL deficiency) [MIM:151670] [P11150 ];

Defects in PNPLA2 are the cause of neutral lipid storage disease with myopathy (NLSDM) [MIM:610717];
also known as neutral lipid storage disease without ichthyosis. NSLDM is a neutral lipid storage disorder (NLSD) with myopathy but without ichthyosis. NLSDs are characterized by the presence of triglyceride-containing cytoplasmic droplets in leukocytes and in other tissues, including bone marrow, skin, and muscle. Individuals with NLSDM did not show obesity, in spite of a defect in triglyceride degradation in fibroblasts and in marked triglyceride storage in liver, muscles, and other visceral cells [Q96AD5 ];

Genetic variations in PNPLA2 may be associated with risk of diabetes mellitus type 2 [Q96AD5 ];

Defects in CEL are a cause of maturity-onset diabetes of the young type 8 with exocrine dysfunction (MODY8) [MIM:609812];
also known as diabetes and pancreatic exocrine dysfunction (DPED). MODY [MIM:606391] is an autosomal dominant form of diabetes mellitus. The pancreas serves both endocrine and exocrine functions. The endocrine cells are found in the islets of Langerhans. They synthesize insulin and other hormones, and are involved in the pathogenesis of diabetes mellitus. The exocrine cells produce bicarbonate and digestive enzymes and are involved in the pathogenesis of pancreatic malabsorption. The localization of the islets within exocrine pancreatic tissue is suggestive of an interdependency and cross-talk between these two cell populations in their normal and in their abnormal function [P19835 ];

Links

SwissProt

P07098; P11150; P16233; P19835; P41247; P54315; P54317; Q17RR3; Q96AD5; Q9NST1; Q9Y5X9

Entrez Gene

1056; 3988; 3990; 5406; 5407; 5408; 80339; 8513; 9388

HPRD

05110; 05109; 02044; 09063; 01058; 04730; 07509; 02005; 02112; 12430



  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min