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HAL is a cytosolic enzyme catalyzing the first reaction in histidine catabolism, the nonoxidative deamination of L-histidine to trans-urocanic acid. HAL defects cause histidinemia which is characterized by increased histidine and histamine and decreased urocanic acid in body fluids Histidine ammonia-lyase is a cytosolic enzyme catalyzing the first reaction in histidine catabolism, the nonoxidative deamination of L-histidine to trans-urocanic acid. Histidine ammonia-lyase defects cause histidinemia which is characterized by increased histidine and histamine and decreased urocanic acid in body fluids.
hal; HIS; histidase; histidine ammonia lyase; histidine ammonia-lyase; histidinemia; Hsd; HSTD; Huth; zgc:136980
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