MAPLE SYRUP DISEASE
- 1 February 1959
- journal article
- Published by American Academy of Pediatrics (AAP)
- Vol. 23 (2) , 348-353
- https://doi.org/10.1542/peds.23.2.348
Abstract
A mixture of alpha-keto acids, the most important of which has been identified as alpha-ketoisocaproic acid, was found to be excreted in large quantities in the urine of beta-methyl-n-valeric acid were present in smaller amounts. Their excretion would suggest an impairment of the degradative metabolism of the branched-chain amino acids as an important cause of the cerebral degeneration in this condition.Keywords
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