Medium-chain triglycerides supplement therapy with a low-carbohydrate formula can supply energy and enhance ammonia detoxification in the hepatocytes of patients with adult–onset type II citrullinemia
Author:
Funder
a grant from the Ministry of Health, Labor and Welfare in Japan
Publisher
Wiley
Subject
Genetics(clinical),Genetics
Link
http://link.springer.com/article/10.1007/s10545-018-0176-1/fulltext.html
Reference21 articles.
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3. Häberle J, Görg B, Rutsch F et al (2005) Congenital glutamine deficiency with glutamine synthetase mutations. N Engl J Med 353:1926–1933
4. Häussinger D (1983) Hepatocyte heterogeneity in glutamine and ammonia metabolism and the role of an intercellular glutamine cycle during ureogenesis in perfused rat liver. Eur J Biochem 133:269–275
5. Häussinger D, Schliess F (2007) Glutamine metabolism and signaling in the liver. Front Biosci 12:371–391
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