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FEBS Lett. 2006 Jun 12;580(14):3551-7. Epub 2006 May 24. PubMed; doi 10.1016/j.febslet.2006.05.040 Brain pyruvate and 2-oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid.Bunik, V. I.; Raddatz, G.; Wanders, R. J.; Reiser, G.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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Почтовый адрес: 119991 г. Москва, ГСП-1, Ленинские горы МГУ 1, стр. 73, Факультет биоинженерии и биоинформатики, комната 433. Телефон / факс: Справочная телефонов МГУ E-mail: bioeng@genebee.msu.ru © 2011 Факультет биоинженерии и биоинформатики Московского Государственного Университета имени М.В.Ломоносова
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