orotic acid การใช้

• OTC Deficiency is also accompanied by hyperammonemia and high orotic acid levels.
• The increased orotic acid concentrations result from the buildup of carbamoyl phosphate.
• Orotic acid can be mutagenic in mammalian somatic cells.
• Here, orotic acid ( one step of this alternative pathway ) levels in the blood are increased.
• These individuals present with hyperammonemia, poor feeding, failure to thrive and increased excretion of orotic acid.
• In addition, in this syndrome, levels of alanine, orotic acid and homocitrulline may be elevated as well.
• Deficiencies of enzymes involved in pyrimidine synthesis can lead to the genetic disease Orotic aciduria which causes excessive excretion of orotic acid in the urine.
• Class 2 DHODHs follow a stepwise mechanism, in which the breaking of the C H bonds precedes the equilibration of iminium into orotic acid.
• Orotic acid is an intermediate product in pyrimidine synthesis pathway, a subsequent product of which plays a role in conversion between dihydrofolate and tetrahydrofolate.
• In people with hyperammonemia orotic acid and homocitrulline appear to be chronically elevated after a high protein diet, but may be normal when fasting.
• "' 4, 5-Dihydroorotic acid "'is a derivative of orotic acid which serves as an intermediate in pyrimidine biosynthesis.
• An individual with untreated OTC deficiency will show decreased citrulline and arginine concentrations ( because the enzyme block is proximal to these intermediates ) and increased orotic acid.
• "' Orotidine "'is a nucleoside formed by attaching orotic acid to a ribose ring via a ?-N 1-glycosidic bond.
• PRPP transferase catalyzes both the ribosylation and decarboxylation reactions, forming UMP from orotic acid in the presence of PRPP . It is from UMP that other pyrimidine nucleotides are derived.
• When this induced enzymatic block is combined with reduced physiologic enzyme activity as seen in heterozygotes, the elevation of orotic acid could be used to differentiate heterozygotes from unaffected individuals.
• This biochemical phenotype ( increased ammonia, low citrulline and increased orotic acid ) is classic for OTC deficiency, but can also be seen in neonatal presentations of ornithine aminotransferase deficiency.
• Tricreatine Orotate is a molecule of creatine bonded to orotic acid, a precursor of the nucleic acids that are used for DNA construction that increases the amount of carnosine in muscle cells.
• In addition to the characteristic excessive orotic acid in the urine, patients typically have megaloblastic anemia ( UMP synthase deficiency ) which cannot be cured by administration of vitamin B12 or folic acid.
• Orotic acid also enhances the formation of creatine phosphate, a source of energy converted to adenosine triphosphate ( ATP ) while the muscle is resting, as well as supporting muscle-protein synthesis.
• IMC has also proven effective in accomplishing enzymatic assays for orotic acid in milk ( Anastasi et al . 2000 ) and malic acid in fruits, wines and other beverages and also cosmetic products ( Antonelli et al . 2008 ).