MAPLE SYRUP URINE DISEASE METABOLITES STUDIED IN CEREBELLUM CULTURES¹

Abstract

Abstract— Myelinating cultures of rat cerebellum were exposed to the branched‐chain amino acids and their α‐keto derivatives at concentrations found in maple syrup urine disease. Decreased‐to‐absent and delayed myelination was produced by α‐keto‐isocaproic acid at physiological levels. Injury to small cells, probably glia, was also noted. The other metabolites failed to produce any visible damage.