Matches in SemOpenAlex for { <https://semopenalex.org/work/W2037628529> ?p ?o ?g. }
Showing items 1 to 80 of
80
with 100 items per page.
- W2037628529 endingPage "930" @default.
- W2037628529 startingPage "921" @default.
- W2037628529 abstract "We investigated the presence of hydroxyacid-oxoacid transhydrogenase (HOT), which catalyses the cofactor-independent conversion of gamma-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." @default.
- W2037628529 created "2016-06-24" @default.
- W2037628529 creator A5001782922 @default.
- W2037628529 creator A5019797356 @default.
- W2037628529 creator A5034654755 @default.
- W2037628529 creator A5052036110 @default.
- W2037628529 creator A5066938409 @default.
- W2037628529 creator A5081248858 @default.
- W2037628529 date "2005-12-01" @default.
- W2037628529 modified "2023-09-27" @default.
- W2037628529 title "Kinetic characterization of human hydroxyacid–oxoacid transhydrogenase: Relevance toD-2-hydroxyglutaric and γ-hydroxybutyric acidurias" @default.
- W2037628529 cites W1499243398 @default.
- W2037628529 cites W1567090673 @default.
- W2037628529 cites W1967658145 @default.
- W2037628529 cites W1976812199 @default.
- W2037628529 cites W1995357041 @default.
- W2037628529 cites W2001028228 @default.
- W2037628529 cites W2001219095 @default.
- W2037628529 cites W2018126373 @default.
- W2037628529 cites W2042296815 @default.
- W2037628529 cites W2045427277 @default.
- W2037628529 cites W2049062289 @default.
- W2037628529 cites W2070810028 @default.
- W2037628529 doi "https://doi.org/10.1007/s10545-005-0114-x" @default.
- W2037628529 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16435184" @default.
- W2037628529 hasPublicationYear "2005" @default.
- W2037628529 type Work @default.
- W2037628529 sameAs 2037628529 @default.
- W2037628529 citedByCount "60" @default.
- W2037628529 countsByYear W20376285292012 @default.
- W2037628529 countsByYear W20376285292013 @default.
- W2037628529 countsByYear W20376285292014 @default.
- W2037628529 countsByYear W20376285292015 @default.
- W2037628529 countsByYear W20376285292016 @default.
- W2037628529 countsByYear W20376285292017 @default.
- W2037628529 countsByYear W20376285292018 @default.
- W2037628529 countsByYear W20376285292019 @default.
- W2037628529 countsByYear W20376285292020 @default.
- W2037628529 countsByYear W20376285292021 @default.
- W2037628529 countsByYear W20376285292022 @default.
- W2037628529 countsByYear W20376285292023 @default.
- W2037628529 crossrefType "journal-article" @default.
- W2037628529 hasAuthorship W2037628529A5001782922 @default.
- W2037628529 hasAuthorship W2037628529A5019797356 @default.
- W2037628529 hasAuthorship W2037628529A5034654755 @default.
- W2037628529 hasAuthorship W2037628529A5052036110 @default.
- W2037628529 hasAuthorship W2037628529A5066938409 @default.
- W2037628529 hasAuthorship W2037628529A5081248858 @default.
- W2037628529 hasConcept C161790260 @default.
- W2037628529 hasConcept C181199279 @default.
- W2037628529 hasConcept C185592680 @default.
- W2037628529 hasConcept C197957613 @default.
- W2037628529 hasConcept C55493867 @default.
- W2037628529 hasConceptScore W2037628529C161790260 @default.
- W2037628529 hasConceptScore W2037628529C181199279 @default.
- W2037628529 hasConceptScore W2037628529C185592680 @default.
- W2037628529 hasConceptScore W2037628529C197957613 @default.
- W2037628529 hasConceptScore W2037628529C55493867 @default.
- W2037628529 hasIssue "6" @default.
- W2037628529 hasLocation W20376285291 @default.
- W2037628529 hasLocation W20376285292 @default.
- W2037628529 hasOpenAccess W2037628529 @default.
- W2037628529 hasPrimaryLocation W20376285291 @default.
- W2037628529 hasRelatedWork W1164537646 @default.
- W2037628529 hasRelatedWork W169735115 @default.
- W2037628529 hasRelatedWork W1986592235 @default.
- W2037628529 hasRelatedWork W2029709235 @default.
- W2037628529 hasRelatedWork W2080203695 @default.
- W2037628529 hasRelatedWork W2084240653 @default.
- W2037628529 hasRelatedWork W2170874906 @default.
- W2037628529 hasRelatedWork W2806378807 @default.
- W2037628529 hasRelatedWork W2925386497 @default.
- W2037628529 hasRelatedWork W950805422 @default.
- W2037628529 hasVolume "28" @default.
- W2037628529 isParatext "false" @default.
- W2037628529 isRetracted "false" @default.
- W2037628529 magId "2037628529" @default.
- W2037628529 workType "article" @default.