We are analyzing https://link.springer.com/article/10.1007/s10545-005-0114-x.

Title:
Kinetic characterization of human hydroxyacid–oxoacid transhydrogenase: Relevance toD-2-hydroxyglutaric and γ-hydroxybutyric acidurias | Journal of Inherited Metabolic Disease
Description:
We investigated the presence of hydroxyacid–oxoacid transhydrogenase (HOT), which catalyses the cofactor-independent conversion of γ-hydroxybutyrate (GHB) to succinic semialdehyde coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG), in human liver extracts employing [2H6]GHB and 2-KG as substrates. We measured incorporation of 2H in D-[2H]2-HG using GC-MS analyses, providing evidence for HOT activity in humans. Kinetic characterization of HOT was undertaken in forward and reverse directions. We employed [2H6]GHB and [2H4]2-KG as cosubstrates in order to develop a HOT activity assay in cultured human fibroblasts derived from patients with D-2-hydroxyglutaric aciduria. HOT activity was quantified in this system by the measurement of D-[2H5]2-HG production. Fibroblasts derived from patients with D-2-hydroxyglutaric aciduria showed normal HOT activities. Our results provide the first demonstration and preliminary kinetic characterization of HOT activity in human tissues.
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Keywords {🔍}

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Topics {✒️}

gamma-aminobutyric acid-transaminase deficiency month download article/chapter inherited metabolic disease hot activity assay human hydroxyacid–oxoacid transhydrogenase d-[2h5]2-hg production d-2-hydroxyglutarate dehydrogenase gene γ-hydroxybutyric acidurias published direct nonisotopic assay d-2-hydroxyglutaric aciduria carrying gc-ms analyses disease-related metabolites neurotransmitter metabolism full article pdf privacy choices/manage cookies employed [2h6]ghb hydroxyacid–oxoacid transhydrogenase hydroxyacid-oxoacid transhydrogenase l-2-hydroxyglutaric acid clinical chemistry related subjects cofactor-independent conversion biochemical marker clinical disease entity γ-hydroxybutyric acidurias human d-2-hgdh d-2-hydroxyglutaric aciduria l-2-hydroxyglutaric aciduria relevance tod-2-hydroxyglutaric l-2-hydroxyglutaric acidemias preliminary kinetic characterization european economic area reverse directions 3-methylglutaconyl-coa hydratase developmental time courses conditions privacy policy medical genetics kaufman ee check access instant access oxidize γ-hydroxybutyrate accepting optional cookies d-[2h]2-hg succinic semialdehyde coupled rat kidney mitochondria article struys article journal journal finder publish main content log craigen

Questions {❓}

Gibson KM, Craigen W, Herman GE, et al (1993a) D-2-Hydroxyglutaric aciduria in a newborn with neurological abnormalities: a new neurometabolic disorder?
Van der Knaap MS, Jakobs C, Hoffmann GF, et al (1999a) D-2-Hydroxyglutaric aciduria: biochemical marker or clinical disease entity?

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