human-PNLIP

Homo sapiens (Human) triacylglycerol lipase (pancreatic lipase)

Comment

Ensembl OTTHUMG00000019103 || Pancreatic lipase (EC 3.1.1.3), a 56-kD protein, is involved in the hydrolysis of dietary triglycerides to fatty acids in the intestine. This hydrolysis, which is initiated in the stomach by gastric lipase, is essential for the intestinal absorption of long-chain triglyceride fatty acids. Pancreatic colipase (120105) is a 12-kD cofactor that appears to overcome the inhibitory effects of intestinal bile salts on pancreatic lipase.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. Failure to thrive has not been observed. Analyses of duodenal contents consistently show a marked decrease of pancreatic lipolytic activity. Fat absorption in patients my may reach 79percent, suggesting that extrapancreatic sources of lipase, such as lingual and gastric lipase, may have an important compensating role in fat digestion. Pancreatic lipase deficiency can be combined with Colipase deficiency

Relationship

Family : Pancreatic_lipase

Block : L

Position in NCBI Life Tree : Homo sapiens

(Below N is a link to NCBI taxonomic web page and E link to ESTHER at designed phylum.)

> cellular organisms N E > Eukaryota N E > Opisthokonta N E > Metazoa N E > Eumetazoa N E > Bilateria N E > Deuterostomia N E > Chordata N E > Craniata N E > Vertebrata N E > Gnathostomata N E > Teleostomi N E > Euteleostomi N E > Sarcopterygii N E > Dipnotetrapodomorpha N E > Tetrapoda N E > Amniota N E > Mammalia N E > Theria N E > Eutheria N E > Boreoeutheria N E > Euarchontoglires N E > Primates N E > Haplorrhini N E > Simiiformes N E > Catarrhini N E > Hominoidea N E > Hominidae N E > Homininae N E > Homo N E > Homo sapiens N E

Database

Sequence

Peptide

MLPLWTLSLL LGAVAGKEVC YERLGCFSDD SPWSGITERP LHILPWSPKD VNTRFLLYTN ENPNNFQEVA ADSSSISGSN FKTNRKTRFI IHGFIDKGEE NWLANVCKNL FKVESVNCIC VDWKGGSRTG YTQASQNIRI VGAEVAYFVE FLQSAFGYSP SNVHVIGHSL GAHAAGEAGR RTNGTIGRIT GLDPAEPCFQ GTPELVRLDP SDAKFVDVIH TDGAPIVPNL GFGMSQVVGH LDFFPNGGVE MPGCKKNILS QIVDIDGIWE GTRDFAACNH LRSYKYYTDS IVNPDGFAGF PCASYNVFTA NKCFPCPSGG CPQMGHYADR YPGKTNDVGQ KFYLDTGDAS NFARWRYKVS VTLSGKKVTG HILVSLFGNK GNSKQYEIFK GTLKPDSTHS NEFDSDVDVG DLQMVKFIWY NNVINPTLPR VGASKIIVET NVGKQFNFCS PETVREEVLL TLTPC

References (34)

Title : Characterization of novel PNLIP variants in congenital pancreatic lipase deficiency - Lin_2023_Pancreatology_S1424-3903_01834
Author(s) : Lin J , Matiwala N , Curry GE , Wilhelm SJ , Cassidy BM , Lowe ME , Xiao X
Ref : Pancreatology , : , 2023
PubMedID: 37926600
Gene_locus related to this paper: human-PNLIP

Title : Identification of protease-sensitive but not misfolding PNLIP variants in familial and hereditary pancreatitis - Masson_2023_Pancreatology__
Author(s) : Masson E , Berthet S , Le Gac G , Le Rhun M , Ka C , Autret S , Gourlaouen I
Ref : Pancreatology , : , 2023
PubMedID: 37270400
Gene_locus related to this paper: human-PNLIP

Title : Novel homozygous mutation of PNLIP gene in congenital pancreatic lipase deficiency: an extended family study - Kamal_2022_Ther.Adv.Chronic.Dis_13_20406223221078757
Author(s) : Kamal NM , Saadah OI , Alheraiti SS , Attar R , Alsufyani AD , El-Shabrawi MHF , Sherief LM
Ref : Ther Adv Chronic Dis , 13 :20406223221078757 , 2022
PubMedID: 35284057
Gene_locus related to this paper: human-PNLIP

Title : Protease-Sensitive Pancreatic Lipase Variants Are Associated With Early Onset Chronic Pancreatitis - Lasher_2019_Am.J.Gastroenterol_114_974
Author(s) : Lasher D , Szabo A , Masamune A , Chen JM , Xiao X , Whitcomb DC , Barmada MM , Ewers M , Ruffert C , Paliwal S , Issarapu P , Bhaskar S , Mani KR , Chandak GR , Laumen H , Masson E , Kume K , Hamada S , Nakano E , Seltsam K , Bugert P , Muller T , Groneberg DA , Shimosegawa T , Rosendahl J , Ferec C , Lowe ME , Witt H , Sahin-Toth M
Ref : Am J Gastroenterol , 114 :974 , 2019
PubMedID: 30789418
Gene_locus related to this paper: human-PNLIP

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 : A novel mutation in PNLIP causes pancreatic triglyceride lipase deficiency through protein misfolding - Szabo_2015_Biochim.Biophys.Acta_1852_1372
Author(s) : Szabo A , Xiao X , Haughney M , Spector A , Sahin-Toth M , Lowe ME
Ref : Biochimica & Biophysica Acta , 1852 :1372 , 2015
PubMedID: 25862608
Gene_locus related to this paper: human-PNLIP

Title : Identification of a novel mutation in the PNLIP gene in two brothers with congenital pancreatic lipase deficiency - Behar_2014_J.Lipid.Res_55_307
Author(s) : Behar DM , Basel-Vanagaite L , Glaser F , Kaplan M , Tzur S , Magal N , Eidlitz-Markus T , Haimi-Cohen Y , Sarig G , Bormans C , Shohat M , Zeharia A
Ref : J Lipid Res , 55 :307 , 2014
PubMedID: 24262094
Gene_locus related to this paper: human-PNLIP

Title : Antiobesity potential of ursolic acid stearoyl glucoside by inhibiting pancreatic lipase - Kazmi_2013_Eur.J.Pharmacol_709_28
Author(s) : Kazmi I , Afzal M , Rahman S , Iqbal M , Imam F , Anwar F
Ref : European Journal of Pharmacology , 709 :28 , 2013
PubMedID: 23500199
Gene_locus related to this paper: human-PNLIP

Title : Analysis of the discriminative inhibition of mammalian digestive lipases by 3-phenyl substituted 1,3,4-oxadiazol-2(3H)-ones. - Point_2012_Eur.J.Med.Chem_58_452
Author(s) : Point V , Pavan Kumar KV , Marc S , Delorme V , Parsiegla G , Amara S , Carriere F , Buono G , Fotiadu F , Canaan S , Leclaire J , Cavalier JF
Ref : Eur Journal of Medicinal Chemistry , 58 :452 , 2012
PubMedID: 23153815
Gene_locus related to this paper: canfa-1lipg , cavpo-2plrp , human-CEL , human-PNLIP , human-PNLIPRP2 , pig-1plip

Title : (-)-Epigallocatechin-3-gallate inhibits pancreatic lipase and reduces body weight gain in high fat-fed obese mice - Grove_2012_Obesity.(Silver.Spring)_20_2311
Author(s) : Grove KA , Sae-tan S , Kennett MJ , Lambert JD
Ref : Obesity (Silver Spring) , 20 :2311 , 2012
PubMedID: 21633405
Gene_locus related to this paper: human-PNLIP

Title : Trp-107 and trp-253 account for the increased steady state fluorescence that accompanies the conformational change in human pancreatic triglyceride lipase induced by tetrahydrolipstatin and bile salt - Bourbon-Freie_2009_J.Biol.Chem_284_14157
Author(s) : Bourbon-Freie A , Dub RE , Xiao X , Lowe ME
Ref : Journal of Biological Chemistry , 284 :14157 , 2009
PubMedID: 19346257
Gene_locus related to this paper: human-PNLIP

Title : Pancreatic lipase and pancreatic lipase-related protein 2, but not pancreatic lipase-related protein 1, hydrolyze retinyl palmitate in physiological conditions - Reboul_2006_Biochim.Biophys.Acta_1761_4
Author(s) : Reboul E , Berton A , Moussa M , Kreuzer C , Crenon I , Borel P
Ref : Biochimica & Biophysica Acta , 1761 :4 , 2006
PubMedID: 16497549
Gene_locus related to this paper: human-PNLIP , human-PNLIPRP2

Title : Val-407 and Ile-408 in the beta5'-loop of pancreatic lipase mediate lipase-colipase interactions in the presence of bile salt micelles - Freie_2006_J.Biol.Chem_281_7793
Author(s) : Freie AB , Ferrato F , Carriere F , Lowe ME
Ref : Journal of Biological Chemistry , 281 :7793 , 2006
PubMedID: 16431912
Gene_locus related to this paper: human-PNLIP

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 : The 2.46 A resolution structure of the pancreatic lipase-colipase complex inhibited by a C11 alkyl phosphonate - Egloff_1995_Biochemistry_34_2751
Author(s) : Egloff MP , Marguet F , Buono G , Verger R , Cambillau C , van Tilbeurgh H
Ref : Biochemistry , 34 :2751 , 1995
PubMedID: 7893686
Gene_locus related to this paper: human-PNLIP

Title : Interfacial activation of the lipase-procolipase complex by mixed micelles revealed by X-ray crystallography - van Tilbeurgh_1993_Nature_362_814
Author(s) : van Tilbeurgh H , Egloff MP , Martinez C , Rugani N , Verger R , Cambillau C
Ref : Nature , 362 :814 , 1993
PubMedID: 8479519
Gene_locus related to this paper: human-PNLIP

Title : The human pancreatic lipase-encoding gene: structure and conservation of an Alu sequence in the lipase gene family - Sims_1993_Gene_131_281
Author(s) : Sims HF , Jennens ML , Lowe ME
Ref : Gene , 131 :281 , 1993
PubMedID: 8406023
Gene_locus related to this paper: human-PNLIP

Title : Structure of the pancreatic lipase-procolipase complex - van Tilbeurgh_1992_Nature_359_159
Author(s) : van Tilbeurgh H , Sarda L , Verger R , Cambillau C
Ref : Nature , 359 :159 , 1992
PubMedID: 1522902
Gene_locus related to this paper: human-PNLIP

Title : Inactivation of gastric and pancreatic lipases by diethyl p-nitrophenyl phosphate - Moreau_1991_Biochemistry_30_1037
Author(s) : Moreau H , Moulin A , Gargouri Y , Noel JP , Verger R
Ref : Biochemistry , 30 :1037 , 1991
PubMedID: 1989675
Gene_locus related to this paper: human-LIPF , human-PNLIP

Title : Assignment of human pancreatic lipase gene (PNLIP) to chromosome 10q24-q26 - Davis_1991_Genomics_11_1164
Author(s) : Davis RC , Diep A , Hunziker W , Klisak I , Mohandas T , Schotz MC , Sparkes RS , Lusis AJ
Ref : Genomics , 11 :1164 , 1991
PubMedID: 1783385
Gene_locus related to this paper: human-PNLIP

Title : Structure of human pancreatic lipase - Winkler_1990_Nature_343_771
Author(s) : Winkler FK , D'Arcy A , Hunziker W
Ref : Nature , 343 :771 , 1990
PubMedID: 2106079
Gene_locus related to this paper: human-PNLIP

Title : Isolated lipase and colipase deficiency in two brothers - Ligumsky_1990_Gut_31_1416
Author(s) : Ligumsky M , Granot E , Branski D , Stankiewicz H , Goldstein R
Ref : Gut , 31 :1416 , 1990
PubMedID: 2265786
Gene_locus related to this paper: human-PNLIP

Title : Cloning and characterization of human pancreatic lipase cDNA - Lowe_1989_J.Biol.Chem_264_20042
Author(s) : Lowe ME , Rosenblum JL , Strauss AW
Ref : Journal of Biological Chemistry , 264 :20042 , 1989
PubMedID: 2479644
Gene_locus related to this paper: human-PNLIP

Title : Isolated congenital lipase-colipase deficiency - Ghishan_1984_Gastroenterology_86_1580
Author(s) : Ghishan FK , Moran JR , Durie PR , Greene HL
Ref : Gastroenterology , 86 :1580 , 1984
PubMedID: 6714581
Gene_locus related to this paper: human-PNLIP

Title : Isolated co-lipase deficiency in two brothers - Hildebrand_1982_Gut_23_243
Author(s) : Hildebrand H , Borgstrom B , Bekassy A , Erlanson-Albertsson C , Helin I
Ref : Gut , 23 :243 , 1982
PubMedID: 7068048
Gene_locus related to this paper: human-PNLIP

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 : [A new case of congenital pancreatic lipase deficiency with presence of colipase (author's transl)] -
Author(s) : Figarella C , de Caro A , Deprez P , Bouvry M , Bernier JJ
Ref : Gastroenterol Clin Biol , 3 :43 , 1979
PubMedID: 437399
Gene_locus related to this paper: human-PNLIP

Title : Proceedings: Studies on the mechanism of fat absorption in congenital isolated lipase deficiency -
Author(s) : Muller DP , McCollum JP , Trompeter RS , Harries JT
Ref : Gut , 16 :838 , 1975
PubMedID: 1205319
Gene_locus related to this paper: human-PNLIP

Title : Presence of colipase in a congenital pancreatic lipase deficiency -
Author(s) : Figarella C , Negri GA , Sarles H
Ref : Biochimica & Biophysica Acta , 280 :205 , 1972
PubMedID: 4627134
Gene_locus related to this paper: human-PNLIP

Title : [Chronic steatorrhea in children due to a congenital insufficiency of pancreatic lipase] -
Author(s) : Verger P , Babin R , Guillard JM , Babin JP , Cixous P , Laigle JL
Ref : Arch Fr Pediatr , 28 :992 , 1971
PubMedID: 5144703
Gene_locus related to this paper: human-PNLIP

Title : Vitamin B 12 malabsorption in chronic pancreatic insufficiency -
Author(s) : Toskes PP , Hansell J , Cerda J , Deren JJ
Ref : N Engl J Med , 284 :627 , 1971
PubMedID: 5547614
Gene_locus related to this paper: human-PNLIP

Title : [Chronic diarrhea due to pancreatic lipase deficiency] -
Author(s) : Larbre F , Hartemann E , Cotton JB , Mathieu M , Charrat A , Moreau P
Ref : Pediatrie , 24 :807 , 1969
PubMedID: 5366614
Gene_locus related to this paper: human-PNLIP

Title : [Congenital absence of pancreatic lipase] -
Author(s) : Rey J , Frezal J , Royer P , Lamy M
Ref : Arch Fr Pediatr , 23 :5 , 1966
PubMedID: 5902779
Gene_locus related to this paper: human-PNLIP

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