Histidine ammonia-lyase
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.
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.
Further reading
References
External links
This article incorporates text from the United States National Library of Medicine, which is in the public domain.
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War machine
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