Neurochemical changes in Leigh's disease.
A series of children with Leigh's disease had normal hepatic pyruvate carboxylase activity, increased cerebral thiamine diphosphate, and decreased cerebral thiamine triphosphate. These thiamine esters were normal in liver. The author suggests that the histologic changes of Leigh's disease, as well as the similar changes of Wernicke's disease, could be due to a deficiency of cerebral thiamine triphosphate.
D000818:Animals; D001921:Brain / Q000378:metabolism; D002490:Central Nervous System / Q000378:metabolism*; D002648:Child; D004678:Encephalomalacia / Q000378:metabolism*; D006801:Humans; D008099:Liver / Q000378:metabolism; D009943:Organophosphorus Compounds; D010744:Phosphoric Monoester Hydrolases / Q000037:antagonists & inhibitors / Q000378:metabolism; D010770:Phosphotransferases / Q000037:antagonists & inhibitors; D011766:Pyruvate Carboxylase / Q000037:antagonists & inhibitors / Q000378:metabolism; D013831:Thiamine / Q000031:analogs & derivatives* / Q000134:cerebrospinal fluid / Q000502:physiology; D013835:Thiamine Pyrophosphate / Q000378:metabolism; D014899:Wernicke Encephalopathy / Q000378:metabolism
D009943:Organophosphorus Compounds; D010770:Phosphotransferases; D010744:Phosphoric Monoester Hydrolases; D011766:Pyruvate Carboxylase; D013835:Thiamine Pyrophosphate; D013831:Thiamine