EFFECTS OF THIAMINE IN A PATIENT WITH A VARIANT FORM OF BRANCHED-CHAIN KETOACIDURIA
Author:
Publisher
Wiley
Subject
General Medicine,Pediatrics, Perinatology and Child Health
Link
http://onlinelibrary.wiley.com/wol1/doi/10.1111/j.1651-2227.1978.tb16336.x/fullpdf
Reference16 articles.
1. BASE EXCESS AND ORGANIC ACIDÆMIA
2. Intermittent Branched-Chain Ketonuria
3. Enzyme activity in classical and variant forms of maple syrup urine disease
4. Thiamine increases the specific activity of human liver branched chain α-ketoacid dehydrogenase
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3. A Novel Regulatory Defect in the Branched-Chain α-Keto Acid Dehydrogenase Complex Due to a Mutation in thePPM1KGene Causes a Mild Variant Phenotype of Maple Syrup Urine Disease;Human Mutation;2012-12-12
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