RT Journal Article
SR Electronic
T1 Pyruvate oxidation in neuromuscular diseases
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP 964
OP 964
DO 10.1212/WNL.24.10.964
VO 24
IS 10
A1 R. A. PIETER KARK
A1 JOHN P. BLASS
A1 W. KING ENGEL
YR 1974
UL http://n.neurology.org/content/24/10/964.abstract
AB To seek clues of metabolic derangements in neurornuscular diseases, the oxidations of pyruvate and succinate were studied in biopsied muscle. Pyruvate oxidation in 7 of 19 spinocerebellar degenerations (0.327± 0.040 prnoles × gm-1 noncollagenous protein content × hr-l) and 8 of 19 motor neuropathies (0.367± 0.029) was less than in controls with myopathic disease (1.096±0.91) or normal muscle (1.707± 0.181) (means± S.E.M.). The rates were independent of several physiologic variables, of the ratios of Type I::Type ll fibers in the specimens, and of the degree of neuropathy. Succinate was oxidized normally. Serially cultured fibroblasts from three patients from families with Friedreich's ataxia also oxidized pyruvate more slowly than did controls (0.11± 0.03 vs. 0.30± 0.02 cpm × mcg-1' protein × hr-1. These two families with ataxia appear to have a genetic defect that affects pyruvate oxidation in some unknown way.