Entrez 3034 | Ensembl ENSG00000084110 | |
External IDs OMIM: 609457 MGI: 96010 HomoloGene: 68229 GeneCards: HAL | ||
Histidine ammonia-lyase (or histidase, or histidinase) is an enzyme that in humans is encoded by the HAL gene. Histidase converts histidine into ammonia and urocanic acid.
Contents
Function
Histidine ammonia-lyase is a cytosolic enzyme catalyzing the first reaction in histidine catabolism, the nonoxidative deamination of L-histidine to trans-urocanic acid. The reaction is catalyzed by an electrophilic co-factor which is formed autocatalytically by cyclization of the protein backbone of the enzyme.
Pathology
Mutations in the gene for histidase are associated with histidinemia and urocanic aciduria.
References
Histidine ammonia-lyase Wikipedia(Text) CC BY-SA