| oroticaciduria |
1. excessive orotic acid in the urine, occurring in several metabolic disorders and also resulting from administration of certain drugs. 2. an autosomal recessive defect of pyrimidine metabolism due to deficiency of orotate phosphoribosyltransferase (OPRT) or orotidine-5′-phosphate decarboxylase (ODC). Manifestations include crystalluria and excessive excretion of orotic acid in the urine, megaloblastic anemia with hypochromic, microcytic circulating erythrocytes, and physical and mental growth retardation. There are two biochemical types: type I is due to deficiency of both OPRT and ODC activities; type II is due to deficiency of ODC activity only.
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| orotidine |
a nucleoside, orotic acid linked by its N1 nitrogen to the C1 carbon of ribose; a phosphorylated derivative, orotidine 5′-phosphate, is an intermediate in pyrimidine nucleotide biosynthesis.
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| orotidine-5’-phosphate decarboxylase |
[EC 4.1.1.23] an enzyme of the lyase class that catalyzes the decarboxylation of orotidine 5′-phosphate to form uridine monophosphate in the synthesis of pyrimidine nucleotides. The catalytic sites for this activity and for the orotate phosphoribosyltransferase (OPRT) activity are on a single protein. Deficiency of ODC activity only, an autosomal recessive trait, results in oroticaciduria, type II.
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| orotidylate |
a dissociated form of orotidylic acid.
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| orotidylate decarboxylase |
orotidine-5′-phosphate decarboxylase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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