argininosuccinate lyase

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ar·gi·ni·no·suc·ci·nate ly·ase

(ar'ji-ni-nō-sŭk'si-nāt lī'ās),
An enzyme cleaving l-argininosuccinate nonhydrolytically to produce l-arginine and fumarate; a deficiency of this enzyme leads to argininosuccinoaciduria; catalyzes a step in the urea cycle.
Synonym(s): argininosuccinase
References in periodicals archive ?
Primary causes of hyperammonemia include congenital enzymopathies in the urea cycle, such as deficiencies of ornithine transcarbamoylase and argininosuccinate lyase.
These include carbamoyl phosphate synthetase I (CPS I) deficiency, N-acetylglutamate synthetase (NAGS) deficiency, ornithine transcarbamylase (OTC) deficiency, argininosuccinate synthetase (ASS) deficiency (which is also known as citrullinemia), argininosuccinate lyase (ASL) deficiency and arginase 1 deficiency (hyperargininemia).
Increased citrulline indicates a deficiency in either argininosuccinate synthase or argininosuccinate lyase (1).
Argininosuccinate lyase deficiency: mutational spectrum in Italian patients and identification of a novel ASL pseudogene.
Argininosuccinic aciduria (ASAuria; OMIM 207900) is an autosomal recessive inborn error of the urea cycle caused by deficiency of the enzyme argininosuccinate lyase (ASL; EC 4.