Affiliation:
1. Division of Pediatric Neurology, Neurological Institute, Columbia-Presbyterian Medical Center
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.
Publisher
American Academy of Pediatrics (AAP)
Subject
Pediatrics, Perinatology and Child Health
Cited by
17 articles.
订阅此论文施引文献
订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献