Catalytic activity:L-arginine + H (2) O = L-ornithine + urea. ,cofactor:Binds 2 manganese ions per subunit. ,Disease:Defects in ARG1 are the cause of argininemia (ARGIN) [MIM:207800]; also known as hyperargininemia. Argininemia is a rare autosomal recessive disorder of the urea cycle. Arginine is elevated in the blood and cerebrospinal fluid , and periodic hyperammonemia occurs. Clinical manifestations include developmental delay , seizures , mental retardation , hypotonia , ataxia , progressive spastic quadriplegia. ,induction:By arginine or homoarginine. ,online information:Arginase entry ,pathway:Nitrogen metabolism; urea cycle; L-ornithine and urea from L-arginine: step 1/1. ,similarity:Belongs to the arginase family. ,subunit:Homotrimer. ,
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