Premium
MAPLE SYRUP URINE DISEASE METABOLITES STUDIED IN CEREBELLUM CULTURES 1
Author(s) -
Silberberg D. H.
Publication year - 1969
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1969.tb05959.x
Subject(s) - maple syrup urine disease , urine , cerebellum , maple , amino acid , biochemistry , metabolite , biology , chemistry , medicine , endocrinology , botany , leucine
— 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.