Source:http://linkedlifedata.com/resource/pubmed/id/16737698
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
14
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pubmed:dateCreated |
2006-6-5
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pubmed:abstractText |
Pyruvate and 2-oxoglutarate dehydrogenase complexes are strongly inhibited by phytanoyl-CoA (IC(50) approximately 10(-6)-10(-7) M). Palmitoyl-CoA is 10-fold less potent. Phytanic or palmitic acids have no inhibitory effect up to 0.3 mM. At the substrate saturation, the acyl-CoA's affect the first and second enzymatic components of the 2-oxoglutarate dehydrogenase complex, while the third component is inhibited only at a low saturation with its substrate dihydrolipoamide. Thus, key regulatory branch points of mitochondrial metabolism are targets of a cellular derivative of phytanic acid. Decreased activity of the complexes might therefore contribute to neurological symptoms upon accumulation of phytanic acid in Refsum disease.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Biological Markers,
http://linkedlifedata.com/resource/pubmed/chemical/Coenzyme A,
http://linkedlifedata.com/resource/pubmed/chemical/Ketoglutarate Dehydrogenase Complex,
http://linkedlifedata.com/resource/pubmed/chemical/Phytanic Acid,
http://linkedlifedata.com/resource/pubmed/chemical/Pyruvic Acid,
http://linkedlifedata.com/resource/pubmed/chemical/phytanoyl-coenzyme A
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pubmed:status |
MEDLINE
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pubmed:month |
Jun
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pubmed:issn |
0014-5793
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:day |
12
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pubmed:volume |
580
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
3551-7
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:16737698-Animals,
pubmed-meshheading:16737698-Biological Markers,
pubmed-meshheading:16737698-Brain,
pubmed-meshheading:16737698-Coenzyme A,
pubmed-meshheading:16737698-Female,
pubmed-meshheading:16737698-Ketoglutarate Dehydrogenase Complex,
pubmed-meshheading:16737698-Models, Molecular,
pubmed-meshheading:16737698-Phytanic Acid,
pubmed-meshheading:16737698-Pyruvic Acid,
pubmed-meshheading:16737698-Rats,
pubmed-meshheading:16737698-Refsum Disease
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pubmed:year |
2006
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pubmed:articleTitle |
Brain pyruvate and 2-oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid.
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pubmed:affiliation |
School of Bioinformatics and Bioengineering and AN Belozersky Institute of Physico-Chemical Biology, Lomonosov Moscow State University, Russian Federation. bunik@belozersky.msu.ru
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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