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Carbamoyl phosphate synthetase I deficiency has an autosomal recessive pattern of inheritance.. CPS I deficiency is inherited in an autosomal recessive manner. [1] This means the defective gene responsible for the disorder is located on an autosome, and two copies of the defective gene (one inherited from each parent) are required in order to be born with the disorder.
"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.
Procollagen-proline dioxygenase, commonly known as prolyl hydroxylase, is a member of the class of enzymes known as alpha-ketoglutarate-dependent hydroxylases.These enzymes catalyze the incorporation of oxygen into organic substrates through a mechanism that requires alpha-Ketoglutaric acid, Fe 2+, and ascorbate.
Isocitrate dehydrogenase (IDH) (EC 1.1.1.42) and (EC 1.1.1.41) is an enzyme that catalyzes the oxidative decarboxylation of isocitrate, producing alpha-ketoglutarate (α-ketoglutarate) and CO 2. This is a two-step process, which involves oxidation of isocitrate (a secondary alcohol ) to oxalosuccinate (a ketone ), followed by the ...
alpha-ketoglutaric acid, a 5-carbon ketoacid derived from glutamic acid. Alpha-ketoglutarate participates in cell signaling by functioning as a coenzyme. [6] It is commonly used in transamination reactions. Beta-keto acids, beta-ketoacids, or 3-oxoacids, such as acetoacetic acid, have the ketone group at the second carbon from the carboxylic acid.
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 ]
Alpha Prime—a company that first specialized in supplement powders like protein, collagen, hydration, and pre-workout—has gone fully viral with their protein brownies called Prime Bites.
α-ketoglutarate + NAD + + CoA → Succinyl CoA + CO 2 + NADH Oxoglutarate dehydrogenase (α-Ketoglutarate dehydrogenase) This reaction proceeds in three steps: decarboxylation of α-ketoglutarate, reduction of NAD + to NADH, and subsequent transfer to CoA, which forms the end product, succinyl CoA. ΔG°' for this reaction is -7.2 kcal mol −1.