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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]
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.
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 .
The Mayo Clinic diet is consistently ranked as one of the best diets according to U.S New and World Report's rankings, coming in fourth for the overall best diet in the 2022 rankings.
13382 Ensembl ENSG00000091140 ENSMUSG00000020664 UniProt P09622 O08749 RefSeq (mRNA) NM_001289752 NM_000108 NM_001289750 NM_001289751 NM_007861 RefSeq (protein) NP_000099 NP_001276679 NP_001276680 NP_001276681 NP_031887 Location (UCSC) Chr 7: 107.89 – 107.93 Mb Chr 12: 31.38 – 31.4 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Dihydrolipoamide dehydrogenase (DLD), also known as ...
The Mayo Clinic diet was created by weight management practitioners at the Mayo Clinic and was designed as a lifestyle change program to promote gradual and sustained weight loss, says Melissa ...
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.
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 ...