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Hyperlysinemia has an autosomal recessive pattern of inheritance. Hyperlysinemia is inherited in an autosomal recessive manner. [2] 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.
α-Ketoglutarate is a component of the citric acid cycle, a cyclical metabolic pathway located in the mitochondria.This cycle supplies the energy that cells need by sequentially metabolizing (indicated by →) citrate through seven intermediate metabolites and then converting the eighth intermediate metabolite, oxaloacetate, back to citrate: [2]
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.
lysine alpha-ketoglutarate reductase deficiency disease; saccharopine dehydrogenase deficiency disease; saccharopinuria; Hyperlysinemia is characterized by elevated plasma lysine levels that exceed 600 μmol/L and can reach up to 2000 μmol/L. [21] [22] These increased levels of lysine do not appear to have any negative effects on the body. [8]
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.
Tolterodine is a longer acting anticholinergic that may have fewer side effects. [9] For urinary retention, cholinergics (muscarinic agonists) like bethanechol can improve the squeezing ability of the bladder. Alpha blockers can also reduce outlet resistance and allow complete emptying if there is adequate bladder muscle function. [9]
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 .
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 ...
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