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An enzyme that catalyzes the first step of histidine catabolism, forming UROCANIC ACID and AMMONIA from HISTIDINE. Deficiency of this enzyme is associated with elevated levels of serum histidine and is called histidinemia (AMINO ACID METABOLISM, INBORN ERRORS).
Entry Term(s)
Histidase
Histidinase
Histidine Deaminase
Histidine alpha-Deaminase
Registry Numbers
EC 4.3.1.3
Related Numbers
9013-75-6
CAS Type 1 Name
L-Histidine ammonia-lyase
Public MeSH Note
98; see HISTIDASE 1991-97, see AMMONIA-LYASES 1973-90
An enzyme that catalyzes the first step of histidine catabolism, forming UROCANIC ACID and AMMONIA from HISTIDINE. Deficiency of this enzyme is associated with elevated levels of serum histidine and is called histidinemia (AMINO ACID METABOLISM, INBORN ERRORS).