PMID:11738042

Citation	Lemke, CT and Howell, PL (2001) The 1.6 A crystal structure of E. coli argininosuccinate synthetase suggests a conformational change during catalysis. Structure 9:1153-64
Abstract	Argininosuccinate synthetase (AS) is the rate-limiting enzyme of both the urea and arginine-citrulline cycles. In mammals, deficiency of AS leads to citrullinemia, a debilitating and often fatal autosomal recessive urea cycle disorder, whereas its overexpression for sustained nitric oxide production via the arginine-citrulline cycle leads to the potentially fatal hypotension associated with septic and cytokine-induced circulatory shock.
Links	PubMed
Keywords	Adenosine Triphosphate/metabolism; Amino Acid Sequence; Animals; Argininosuccinate Synthase/chemistry; Argininosuccinate Synthase/genetics; Aspartic Acid/chemistry; Binding Sites; Catalysis; Citrulline/chemistry; Crystallography, X-Ray; Dimerization; Escherichia coli/enzymology; Humans; Models, Chemical; Models, Molecular; Molecular Sequence Data; Mutation; Nucleotides/metabolism; Protein Conformation; Protein Structure, Secondary; Protein Structure, Tertiary; Sequence Homology, Amino Acid
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