Abstract
The incorporation of [1- 14C]propionate into trichloroacetic acid-precipitable material was found to be greater in normal fibroblasts than in cells cultured from patients with PrAE and MMAU. In MMAU cells, the metabolic pathway leading into the TCA cycle appeared to be completely blocked. In PrAE cells, however, trace amounts of labeled TCA cycle intermediates and lactic acid were detected, indicating an incomplete block in the propionate pathway. A large accumulation of labeled long-chain fatty acids by affected cell lines points to a possible role of fatty acids in pathogenesis of these disorders.
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