DPYS polyclonal antibody

DPYS (dihydropyrimidinase), also known as DHPase, Hydantoinase or DHP, is a 519 amino acid protein that is expressed in liver and kidney tissue and belongs to the DHOase family. Functioning as a homotetramer, DPYS uses zinc as a cofactor to catalyze the second step of reductive pyrimidine degradation, namely the conversion of 5,6-dihydrouracil to 3-ureidopropionate. DPYS is subject to post-translational carbamylation, an event which enhances the ability of DPYS to bind zinc ions. Defects in the gene encoding DPYS are the cause of DHP deficiency, an autosomal recessive disorder that is characterized by epileptic or convulsive attacks, dysmorphic features and severe developmental delay and congenital microvillous atrophy.