title: Elevated plasma concentrations of the endogenous nitric oxide synthase inhibitor asymmetric dimethylarginine in citrullinemia.
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.
authors.affiliation: Department of Pediatrics, Medical School of Hannover, Carl Neuberg Str. 1, D-30625 Hannover, Germany. luecke.thomas@mh-hannover.de
authors.forename: Thomas
authors.initials: T
authors.lastname: Lücke
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Tsikas
authors.forename: Nele
authors.initials: N
authors.lastname: Kanzelmeyer
authors.forename: Bernhard
authors.initials: B
authors.lastname: Vaske
authors.forename: Anibh Martin
authors.initials: AM
authors.lastname: Das
mesh_terms: D001120:Arginine / Q000031:analogs & derivatives* / Q000097:blood / Q000502:physiology / Q000652:urine; D020159:Citrullinemia / Q000097:blood* / Q000209:etiology; D004791:Enzyme Inhibitors / Q000097:blood*; D006801:Humans; D009569:Nitric Oxide / Q000502:physiology; D019001:Nitric Oxide Synthase / Q000037:antagonists & inhibitors*
chemical_list: D004791:Enzyme Inhibitors; D009569:Nitric Oxide; C018524:N,N-dimethylarginine; D001120:Arginine; D019001:Nitric Oxide Synthase