Pancreatic, hepatic and gastric/lingual lipase are closely related to each other and to lipoprotein lipase (EC: 3.1.1.34), which hydrolyses triglycerides of chylomicrons and very low density lipoproteins (VLDL). Pancreatic lipase (triacylglycerol acylhydrolase, EC: 3.1.1.3) plays a key role in dietary fat absorption by hydrolysing dietary long chain triacyl-glycerol to free fatty acids and monoacylglycerols in the intestinal lumen. The activity of lipase is stimulated by colipase in the presence of bile acids. Congenital pancreatic lipase deficiency is a rare, monoenzymatic form of exocrine pancreatic failure. Patients have oily/greasy stools from infancy or early childhood and the absence of discernable pancreatic disease. The pancreatic lipase-related protein show no significant catalytic activity on any of the substrates tested di and tri-glycerides phospholipids. Introducing the double mutation Val 178 Ala and Ala 180 Pro into the human pancreatic RP1 HPLRP1 gene yielded an enzyme is kinetically active on triglycerides. The guinea pig pancreatic lipase-related protein 2 (GPLRP2) differs from classical pancreatic lipases in that it displays both lipase and phospholipase A1 activities; classical pancreatic lipases have no phospholipase activity. human-PNLIPRP2 adopt in solution an open lid conformation which creates a large cavity capable of accommodating the galactose polar head of galactolipids (galactolipase)
Interpro : IPR000734 Lipase , IPR002331 Pancreatic lipase , IPR016272 Lipase, LIPH-type , IPR013818 Lipase
PIRSF : PIRSF000865 lipoprotein lipase, LIPH type
Pdoc : PDOC00110
Pfam : PF00151 Lipase
Prints : PR00821 , PR00823
EC Number : 3.1.1.3
Structures (12)
Genes Proteins in Pancreatic_lipase family (488)
Fragments of genes in Pancreatic_lipase family (103)
Structures in Pancreatic_lipase family (12)Substrates in Pancreatic_lipase family (18)
Inhibitors in Pancreatic_lipase family (15)
Title : Comparative Study of the Molecular Characterization, Evolution, and Structure Modeling of Digestive Lipase Genes Reveals the Different Evolutionary Selection Between Mammals and Fishes - Tang_2022_Front.Genet_13_909091 |
Author(s) : Tang SL , Liang XF , He S , Li L , Alam MS , Wu J |
Ref : Front Genet , 13 :909091 , 2022 |
PubMedID: 35991544 |
Title : Pancreatic lipase inhibitors: The road voyaged and successes - Kumar_2021_Life.Sci__119115 |
Author(s) : Kumar A , Chauhan S |
Ref : Life Sciences , :119115 , 2021 |
PubMedID: 33515565 |
Title : Structure and Function of Pancreatic Lipase-Related Protein 2 and Its Relationship With Pathological States - Zhu_2021_Front.Genet_12_693538 |
Author(s) : Zhu G , Fang Q , Zhu F , Huang D , Yang C |
Ref : Front Genet , 12 :693538 , 2021 |
PubMedID: 34290745 |
Gene_locus related to this paper: human-PNLIPRP2 |
Title : The beta5-Loop and Lid Domain Contribute to the Substrate Specificity of Pancreatic Lipase-related Protein 2 (PNLIPRP2) - Xiao_2015_J.Biol.Chem_290_28847 |
Author(s) : Xiao X , Lowe ME |
Ref : Journal of Biological Chemistry , 290 :28847 , 2015 |
PubMedID: 26494624 |
Gene_locus related to this paper: human-PNLIP , human-PNLIPRP2 |
Title : Inhibition of phospholipase A1, lipase and galactolipase activities of pancreatic lipase-related protein 2 by methyl arachidonyl fluorophosphonate (MAFP) - Amara_2012_Biochim.Biophys.Acta_1821_1379 |
Author(s) : Amara S , Delorme V , Record M , Carriere F |
Ref : Biochimica & Biophysica Acta , 1821 :1379 , 2012 |
PubMedID: 22835523 |
Gene_locus related to this paper: human-PNLIPRP2 |
Title : Structure of human pancreatic lipase-related protein 2 with the lid in an open conformation - Eydoux_2008_Biochemistry_47_9553 |
Author(s) : Eydoux C , Spinelli S , Davis TL , Walker JR , Seitova A , Dhe-Paganon S , de Caro A , Cambillau C , Carriere F |
Ref : Biochemistry , 47 :9553 , 2008 |
PubMedID: 18702514 |
Gene_locus related to this paper: human-PNLIPRP2 |
Title : Human pancreatic lipase-related protein 2 is a galactolipase - Sias_2004_Biochemistry_43_10138 |
Author(s) : Sias B , Ferrato F , Grandval P , Lafont D , Boullanger P , de Caro A , Leboeuf B , Verger R , Carriere F |
Ref : Biochemistry , 43 :10138 , 2004 |
PubMedID: 15287741 |
Gene_locus related to this paper: human-PNLIPRP2 |
Title : Reactivation of the totally inactive pancreatic lipase RP1 by structure-predicted point mutations - Roussel_1998_Proteins_32_523 |
Author(s) : Roussel A , De Caro J , Bezzine S , Gastinel L , de Caro A , Carriere F , Leydier S , Verger R , Cambillau C |
Ref : Proteins , 32 :523 , 1998 |
PubMedID: 9726421 |
Gene_locus related to this paper: canfa-1plip |
Title : A pancreatic lipase with a phospholipase A1 activity: crystal structure of a chimeric pancreatic lipase-related protein 2 from guinea pig - Withers-Martinez_1996_Structure_4_1363 |
Author(s) : Withers-Martinez C , Carriere F , Verger R , Bourgeois D , Cambillau C |
Ref : Structure , 4 :1363 , 1996 |
PubMedID: 8939760 |
Gene_locus related to this paper: human-PNLIP |
Title : Two novel human pancreatic lipase related proteins, hPLRP1 and hPLRP2. Differences in colipase dependence and in lipase activity - Giller_1992_J.Biol.Chem_267_16509 |
Author(s) : Giller T , Buchwald P , Blum-Kaelin D , Hunziker W |
Ref : Journal of Biological Chemistry , 267 :16509 , 1992 |
PubMedID: 1379598 |
Gene_locus related to this paper: human-LPL , human-PNLIPRP1 , human-PNLIPRP2 |
Title : Congenital pancreatic lipase deficiency - Figarella_1980_J.Pediatr_96_412 |
Author(s) : Figarella C , de Caro A , Leupold D , Poley JR |
Ref : J Pediatr , 96 :412 , 1980 |
PubMedID: 6153713 |
Gene_locus related to this paper: human-PNLIP , human-PNLIPRP1 |
Title : CONGENITAL PANCREATIC LIPASE DEFICIENCY - |
Author(s) : Sheldon W |
Ref : Arch Dis Child , 39 :268 , 1964 |
PubMedID: 14169458 |
Gene_locus related to this paper: human-PNLIP |