Biochemical studies in mitochondrial encephalomyopathy.
The alpha-keto acid dehydrogenase complex and its component enzymes, lactate dehydrogenase, pyruvate carboxylase, cytochrome c oxidase, succinate-cytochrome c reductase, NADH-cytochrome c reductase, and the concentration of cytochromes and enzymes of beta-oxidation in muscle from a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes were studied and no specific defect was found. These results raise the possibility that the mitochondrial changes in the patient may be secondary.
Goda
S
S
Department of Neurology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Ishimoto
S
S
Goto
I
I
Kuroiwa
Y
Y
Koike
K
K
Koike
M
M
Nakagawa
M
M
Reichmann
H
H
DiMauro
S
S
D000140:Acidosis, Lactic / Q000150:complications* / Q000201:enzymology / Q000473:pathology; D000293:Adolescent; D001927:Brain Diseases / Q000150:complications* / Q000201:enzymology / Q000473:pathology; D002561:Cerebrovascular Disorders / Q000150:complications* / Q000201:enzymology / Q000473:pathology; D005260:Female; D006801:Humans; D008931:Mitochondria, Muscle / Q000201:enzymology / Q000648:ultrastructure*; D009135:Muscular Diseases / Q000150:complications* / Q000201:enzymology / Q000473:pathology; D013577:Syndrome