argininosuccinate synthase


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argininosuccinate synthase

/ar·gi·ni·no·suc·cin·ate syn·thase/ (sin´thās) an enzyme that catalyzes the condensation of citrulline and aspartate, a step in the hepatic urea cycle; deficiency is an inherited disorder characterized by elevated plasma levels of citrulline and ammonia and urinary excretion of citrulline and orotic acid, often with mental retardation and neurologic abnormalities.

ASS1

A gene on chromosome 9q34.1 that encodes an enzyme which catalyses the penultimate step of the arginine biosynthetic pathway.
 
Molecular pathology
ASS1 mutations cause citrullinemia type 1.
References in periodicals archive ?
A majority of human pancreatic adenocarcinomas are deficient in argininosuccinate synthase (ASS) and are therefore sensitive to arginine deprivation by ADI-PEG 20.
liver-type arginase (ARG), ornithine carbamoyltransferase (OCT), and argininosuccinate synthase (AS), exist almost exclusively in the liver (1-3) and may serve as more specific markers of liver injury.