Elevated plasma concentrations of the endogenous nitric oxide synthase inhibitor asymmetric dimethylarginine in citrullinemia.
Metabolism
; 55(12): 1599-603, 2006 Dec.
Article
em En
| MEDLINE
| ID: mdl-17142130
ABSTRACT
Citrullinemia is an inborn error of the urea cycle with deficiency of the argininosuccinate synthase. It is characterized by elevated concentrations of l-citrulline and decreased levels of l-arginine in body fluids. Asymmetric dimethylarginine is an endogenous inhibitor of nitric oxide synthase that converts l-arginine to l-citrulline and nitric oxide (NO). Asymmetric dimethylarginine is hydrolyzed by the enzyme dimethylarginine dimethylaminohydrolase to l-citrulline and dimethylamine. Elevation of l-citrulline in citrullinemia prompted us to study the l-arginine/NO pathway in this disorder. In 8 children with citrullinemia (3 days to 3 years of age), elevated plasma levels of asymmetric dimethylarginine (P = .028) were found compared with age-matched healthy children. We hypothesize that the l-arginine/NO pathway plays a role in the pathophysiology of citrullinemia.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Arginina
/
Óxido Nítrico Sintase
/
Citrulinemia
/
Inibidores Enzimáticos
Tipo de estudo:
Etiology_studies
Limite:
Humans
Idioma:
En
Revista:
Metabolism
Ano de publicação:
2006
Tipo de documento:
Article
País de afiliação:
Alemanha