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Various organic compounds consisting of carbon, hydrogen, and oxygen atoms that are found in foods and living tissues; typically broken down to release energy; includes sugars, sugar alcohols, sugar acids and derivatives, glycosyl compounds, and more.
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DL- -Hydroxyglutaric acid disodium salt (CAS 40951-21-1) is a small molecule analogue of -hydroxyglutaric acid a metabolite implicated in neurometabolic disorders In humans aberrant accumulation of D- -hydroxyglutaric acid occurs in D-2-hydroxyglutaric aciduria associated with mutations in isocitrate dehydrogenase (IDH) enzymes particularly IDH1 and IDH2 These mutations alter normal metabolic flux leading to overproduction of D- -hydroxyglutaric acid from 2-oxoglutarate In preclinical studies elevated levels of this metabolite are detectable in IDH1-mutant glioma models and non-invasive quantification is feasible via magnetic resonance spectroscopy DL- -Hydroxyglutaric acid disodium salt is utilized in research investigating metabolic dysregulation biomarker development and disease mechanisms linked to IDH mutations and gliomagenesis
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