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"Ketoglutaric acid" and "ketoglutarate", when not qualified as α or β, almost always refers respectively to α-ketoglutaric acid or α-ketoglutarate. [2] α-Ketoglutarate is an intermediate in the citric acid cycle, a cycle that supplies the energy to cells. [2] It is also an intermediate in or product of several other metabolic pathways.
The deficiency leads to decreased activity in the intrinsic pathway (F-IX) factors, monitored by PTT, and the extrinsic pathway (F-VII) which PT monitors. However, factor VII has the shortest half-life of all the factors carboxylated by vitamin K; therefore, when deficient, it is the PT that rises first, since the activated Factor VII is the ...
Calcium-alpha-ketoglutarate (C 5 H 4 CaO 5 •H 2 O) is a special form mineral calcium that can be used to restore calcium concentration level in the blood back to normal. Calcium-alpha-ketoglutarate binds excess phosphate and pass it as a waste, re-establishing normal balance of calcium and phosphate in the body.
This gene encodes a subunit that catalyzes the oxidative decarboxylation of alpha-ketoglutarate to Succinyl-CoA at its active site in the fourth step of the citric acid cycle by acting as a base to facilitate the decarboxylation. The main residues responsible for the catalysis are thought to be His 260, Phe 227, Gln685, His 729, Ser302, and His ...
A deficiency in any of the enzymes of this complex as well as an inhibition of the complex as a whole leads to a buildup of branched-chain amino acids and their harmful derivatives in the body. These accumulations lend a sweet smell to bodily excretions (such as ear wax and urine), leading to a pathology known as maple syrup urine disease .
Fat mass and obesity-associated protein, also known as alpha-ketoglutarate-dependent dioxygenase FTO, is an enzyme that in humans is encoded by the FTO gene located on chromosome 16. As one homolog in the AlkB family proteins, it is the first messenger RNA (mRNA) demethylase that has been identified. [ 5 ]
The disease results from a deficiency in hepatic tyrosine aminotransferase. [10] Tyrosinemia type II (Richner-Hanhart syndrome, RHS) is a disease of autosomal recessive inheritance characterized by keratitis, palmoplantar hyperkeratosis, mental retardation, and elevated blood tyrosine levels. [10]
Mutations in isocitrate dehydrogenase (IDH1 and IDH2), which frequently occur in glioma and AML, [1] [2] [3] produce D-2-hydroxyglutarate from alpha-ketoglutarate. [4] D-2-hydroxyglutarate accumulates to very high concentrations which inhibits the function of enzymes that are dependent on alpha-ketoglutarate, including histone lysine demethylases.