pubmed-article:19801371 | rdf:type | pubmed:Citation | lld:pubmed |
pubmed-article:19801371 | lifeskim:mentions | umls-concept:C0009235 | lld:lifeskim |
pubmed-article:19801371 | lifeskim:mentions | umls-concept:C1427541 | lld:lifeskim |
pubmed-article:19801371 | lifeskim:mentions | umls-concept:C0126485 | lld:lifeskim |
pubmed-article:19801371 | pubmed:issue | 4 | lld:pubmed |
pubmed-article:19801371 | pubmed:dateCreated | 2010-3-10 | lld:pubmed |
pubmed-article:19801371 | pubmed:abstractText | 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. | lld:pubmed |
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pubmed-article:19801371 | pubmed:language | eng | lld:pubmed |
pubmed-article:19801371 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:19801371 | pubmed:citationSubset | IM | lld:pubmed |
pubmed-article:19801371 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
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pubmed-article:19801371 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
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pubmed-article:19801371 | pubmed:status | MEDLINE | lld:pubmed |
pubmed-article:19801371 | pubmed:month | Apr | lld:pubmed |
pubmed-article:19801371 | pubmed:issn | 0022-2275 | lld:pubmed |
pubmed-article:19801371 | pubmed:author | pubmed-author:StorchJudithJ | lld:pubmed |
pubmed-article:19801371 | pubmed:author | pubmed-author:XuZhiZ | lld:pubmed |
pubmed-article:19801371 | pubmed:author | pubmed-author:CarmanGeorge... | lld:pubmed |
pubmed-article:19801371 | pubmed:author | pubmed-author:SubramanianVi... | lld:pubmed |
pubmed-article:19801371 | pubmed:author | pubmed-author:BrasaemleDawn... | lld:pubmed |
pubmed-article:19801371 | pubmed:author | pubmed-author:CavigliaJorge... | lld:pubmed |
pubmed-article:19801371 | pubmed:author | pubmed-author:RothenbergAle... | lld:pubmed |
pubmed-article:19801371 | pubmed:author | pubmed-author:Montero-Moran... | lld:pubmed |
pubmed-article:19801371 | pubmed:author | pubmed-author:McMahonDerekD | lld:pubmed |
pubmed-article:19801371 | pubmed:author | pubmed-author:Lara-Gonzalez... | lld:pubmed |
pubmed-article:19801371 | pubmed:issnType | Print | lld:pubmed |
pubmed-article:19801371 | pubmed:volume | 51 | lld:pubmed |
pubmed-article:19801371 | pubmed:owner | NLM | lld:pubmed |
pubmed-article:19801371 | pubmed:authorsComplete | Y | lld:pubmed |
pubmed-article:19801371 | pubmed:pagination | 709-19 | lld:pubmed |
pubmed-article:19801371 | pubmed:dateRevised | 2011-7-27 | lld:pubmed |
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