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- W2090910975 abstract "In BCKA, leucine (Leu) metabolites are generally considered the cause of the toxic encephalopathy. An infant with intermittent (I) BCKA (thiamine unresponsive) demonstrated severe CNS toxicity to valine (val). This child had marked symptoms with serum leu levels <7.0 mg%; 7 other children with classic (C)-BCKA had no acute symptoms with serum leu <10 mg%. Oral load tests (100 mg/kg) with leu, val and iso-leucine (i-leu) in 2 children with C-BCKA caused no symptoms; the same tests in the I-BCKA child caused severe transient obtundation, EEG changes, lactic acidosis and hypoglycemia with val but no effects with leu or i-leu. Peak serum levels for each amino acid were similar in all children and always >16.0 mg%. Cultured fibroblast from children with C-BCKA show increasing decarboxylase activity with increasing substrate concentrations for Leu-1-14C and Val-1-14C. In fibroblast cultures from the I-BCKA child, the leu response was similar, but different with valine: This variant form of BCKA has a unique neuronal toxicity to val not previously reported that correlates with in-vitro studies." @default.
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- W2090910975 date "1977-04-01" @default.
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- W2090910975 title "VALINE TOXICITY IN INTERMITTENT BRANCHED-CHAIN KETO- ACIDURIA (BCKA) WITH ATYPICAL DECARBOXYLASE ACTIVITY" @default.
- W2090910975 doi "https://doi.org/10.1203/00006450-197704000-00577" @default.
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