Argininosuccinate lyase

Definition:

Catalyzes the reversible cleavage of L-argininosuccinate to fumarate and L-arginine, an intermediate step reaction in the urea cycle mostly providing for hepatic nitrogen detoxification into excretable urea as well as de novo L-arginine synthesis in nonhepatic tissues. Essential regulator of intracellular and extracellular L-arginine pools. As part of citrulline-nitric oxide cycle, forms tissue-specific multiprotein complexes with argininosuccinate synthase ASS1, transport protein SLC7A1 and nitric oxide synthase NOS1, NOS2 or NOS3, allowing for cell-autonomous L-arginine synthesis while channeling extracellular L-arginine to nitric oxide synthesis pathway.

References:

[1]. Ayelet Erez, et al. Requirement of argininosuccinate lyase for systemic nitric oxide production. Nat Med. 2011 Nov 13;17(12):1619-26. [Content Brief]

[2]. B Yu, et al. Mechanisms for intragenic complementation at the human argininosuccinate lyase locus. Biochemistry. 2001 Dec 25;40(51):15581-90. [Content Brief]

[3]. L M Sampaleanu, et al. Three-dimensional structure of the argininosuccinate lyase frequently complementing allele Q286R. Biochemistry. 2001 Dec 25;40(51):15570-80. [Content Brief]

[4]. D C Walker, et al. Intragenic complementation at the human argininosuccinate lyase locus. Identification of the major complementing alleles. J Biol Chem. 1997 Mar 7;272(10):6777-83. [Content Brief]

[5]. D C Walker, et al. Molecular analysis of human argininosuccinate lyase: mutant characterization and alternative splicing of the coding region. Proc Natl Acad Sci U S A. 1990 Dec;87(24):9625-9. [Content Brief]

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