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Histidine Ammonia-Lyase

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Definition: 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).  /defic: consider also histidinemia (AMINO ACID METABOLISM, INBORN ERRORS)    Other names Histidase; alpha-Deaminase, Histidine; Histidine alpha Deaminase; Histidine Ammonia Lyase; Deaminase, Histidine; Ammonia-Lyase, Histidine; Histidine alpha-Deaminase; Histidine Deaminase; Histidinase
 
SubstanceCAS Registry & nameCategoriesSourceDrugs*
Histidinemia  0   *Amino Acid Metabolism, Inborn Errors *Intellectual Disability Histidine Ammonia-Lyase/deficiency.
1-amino-2-(imidazol-4'-yl)ethylphosphonic acid  156721-55-0   *Phosphonic Acids Histidine/*analogs & derivatives Histidine Ammonia-Lyase/antagonists & inhibitors. Biochem Mol Biol Int 1994 Jan;32(1):189-94

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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