NDLI: CGI-58/ABHD5 is a coenzyme A-dependent lysophosphatidic acid acyltransferase.

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CGI-58/ABHD5 is a coenzyme A-dependent lysophosphatidic acid acyltransferase.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Montero-Moran, Gabriela Caviglia, Jorge M. McMahon, Derek Rothenberg, Alexis Subramanian, Vidya Xu, Zhi Lara-Gonzalez, Samuel Storch, Judith Carman, George M. Brasaemle, Dawn L.
Description	Country affiliation: United States Author Affiliation: Montero-Moran G ( Rutgers Center for Lipid Research, Rutgers, The State University of New Jersey, New Brunswick, NJ 08901, USA.)
Abstract	Mutations in human CGI-58/ABHD5 cause Chanarin-Dorfman syndrome (CDS), characterized by excessive storage of triacylglycerol in tissues. CGI-58 is an alpha/beta-hydrolase fold enzyme expressed in all vertebrates. The carboxyl terminus includes a highly conserved consensus sequence (HXXXXD) for acyltransferase activity. Mouse CGI-58 was expressed in Escherichia coli as a fusion protein with two amino terminal 6-histidine tags. Recombinant CGI-58 displayed acyl-CoA-dependent acyltransferase activity to lysophosphatidic acid, but not to other lysophospholipid or neutral glycerolipid acceptors. Production of phosphatidic acid increased with time and increasing concentrations of recombinant CGI-58 and was optimal between pH 7.0 and 8.5. The enzyme showed saturation kinetics with respect to 1-oleoyl-lysophosphatidic acid and oleoyl-CoA and preference for arachidonoyl-CoA and oleoyl-CoA. The enzyme showed slight preference for 1-oleoyl lysophosphatidic acid over 1-palmitoyl, 1-stearoyl, or 1-arachidonoyl lysophosphatidic acid. Recombinant CGI-58 showed intrinsic fluorescence for tryptophan that was quenched by the addition of 1-oleoyl-lysophosphatidic acid, oleoyl-CoA, arachidonoyl-CoA, and palmitoyl-CoA, but not by lysophosphatidyl choline. Expression of CGI-58 in fibroblasts from humans with CDS increased the incorporation of radiolabeled fatty acids released from the lipolysis of stored triacylglycerols into phospholipids. CGI-58 is a CoA-dependent lysophosphatidic acid acyltransferase that channels fatty acids released from the hydrolysis of stored triacylglycerols into phospholipids.
File Format	HTM / HTML
ISSN	00222275
e-ISSN	15397262
DOI	10.1194/jlr.M001917
Journal	The Journal of Lipid Research
Issue Number	4
Volume Number	51
Language	English
Publisher	American Society for Biochemistry and Molecular Biology
Publisher Date	2010-04-01
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Discipline Biochemistry 1-acylglycerol-3-phosphate O-acyltransferase Metabolism Acyl Coenzyme A Acyltransferases Lysophospholipids Chemistry Genetics Isolation & Purification Amino Acid Motifs Animals Cells, Cultured Gene Expression Hydrogen-ion Concentration Kinetics Lipid Metabolism Lipid Metabolism, Inborn Errors Enzymology Mice Position-specific Scoring Matrices Protein Binding Recombinant Proteins Substrate Specificity Syndrome Research Support, N.i.h., Extramural Research Support, Non-u.s. Gov't
Content Type	Text
Resource Type	Article
Subject	Cell Biology Biochemistry Endocrinology

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CGI-58, the causative gene for Chanarin-Dorfman syndrome, mediates acylation of lysophosphatidic acid.

CGI-58/ABHD5 is a coenzyme A-dependent lysophosphatidic acid acyltransferase

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ABHD5/CGI-58 facilitates the assembly and secretion of apolipoprotein B lipoproteins by McA RH7777 rat hepatoma cells.

CGI-58/ABHD5 is a coenzyme A-dependent lysophosphatidic acid acyltransferase.

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CGI-58/ABHD5 is a coenzyme A-dependent lysophosphatidic acid acyltransferase.