Reduction of large neutral amino acid concentrations in plasma and CSF of patients with maple syrup urine disease during crises
Author:
Affiliation:
1. ; Serviço de Genética Médica; Hospital de Clínicas de Porto Alegre; Porto Alegre RS Brazil
2. Departamento de Bioquímica; Instituto de Ciências Básicas de Saúde, UFRGS; Porto Alegre, RS Brazil
Publisher
Wiley
Subject
Genetics(clinical),Genetics
Link
http://onlinelibrary.wiley.com/wol1/doi/10.1023/A:1005668431926/fullpdf
Reference28 articles.
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3. Leucine-a possible regulator of protein turnover in muscle;Buse;J Clin Invest,1975
4. Evidence for inhibition of exodus of small neutral amino acids from non-brain tissues in hyperphenylalaninaemia rats;Cespedes;J Inherit Metab Dis,1989
5. Where do the depleted plasma amino acids go in phenylketonuria?;Christensen;Biochem J,1986
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