Article
Increased anaerobic glycolysis in mitochondrial trifunctional protein-deficient brain.
Journal of the neurological sciences - 1 Mar 2001
Miyajima H, Ouchi Y, Sakamoto M, Takahashi Y, Kono S, Suzuki H
Abstract excerpt
Deficiency of mitochondrial trifunctional protein (TP), beta-oxidation enzyme, is characterized by recurrent rhabdomyolysis in adult patients. Positron emission tomography was used to measure brain oxygen (CMRO(2)) and glucose (CMRGlc) metabolisms in an adult patient with TP deficiency who had a homozygous G1331A transition of the beta-subunit gene. The molar ratio of oxygen to glucose consumption showed diffuse...
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