ArticleJournal of inherited metabolic disease2007
Novel insights into L-2-hydroxyglutaric aciduria: mass isotopomer studies reveal 2-oxoglutaric acid as the metabolic precursor of L-2-hydroxyglutaric acid.
Article in Journal of inherited metabolic disease, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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21 citing papers in PubMed, 30 citations in OpenAlex.
- Salidroside and mesenchymal stem cells alleviate hypobaric hypoxia-induced lung injury and are associated with alterations in the gut-lung axis.Biochemistry and biophysics reports · 2026Article
- Moyamoya syndrome in a patient with D-2-hydroxyglutaric aciduria type II: a rare association.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Article
- Detection and analysis of chiral molecules as disease biomarkers.Nature reviews. Chemistry · 2023Review
- MYC Regulation of D2HGDH and L2HGDH Influences the Epigenome and Epitranscriptome.Cell chemical biology · 2020Article
- Findings on serial MRI in a childhood case of L2-hydroxyglutaric aciduria.Radiology case reports · 2020Article
- Biochemical and Epigenetic Insights into L-2-Hydroxyglutarate, a Potential Therapeutic Target in Renal Cancer.Clinical cancer research : an official journal of the American Association for Cancer Research · 2018Article
- Metabolism, Activity, and Targeting of D- and L-2-Hydroxyglutarates.Trends in cancer · 2018Review
- Saccharomyces cerevisiae Forms D-2-Hydroxyglutarate and Couples Its Degradation to D-Lactate Formation via a Cytosolic Transhydrogenase.The Journal of biological chemistry · 2016Article
- Hypoxia-Mediated Increases in L-2-hydroxyglutarate Coordinate the Metabolic Response to Reductive Stress.Cell metabolism · 2015Article
- Article
- L-2-Hydroxyglutarate: an epigenetic modifier and putative oncometabolite in renal cancer.Cancer discovery · 2014Article
- Metabolite proofreading, a neglected aspect of intermediary metabolism.Journal of inherited metabolic disease · 2013Review
- A L2HGDH initiator methionine codon mutation in a Yorkshire terrier with L-2-hydroxyglutaric aciduria.BMC veterinary research · 2012Article
- Progress in understanding 2-hydroxyglutaric acidurias.Journal of inherited metabolic disease · 2012Review
- Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading.The Journal of biological chemistry · 2011Article
- The sodium-dependent di- and tricarboxylate transporter, NaCT, is not responsible for the uptake of D-, L-2-hydroxyglutarate and 3-hydroxyglutarate into neurons.Journal of inherited metabolic disease · 2011Article
- Plant D-2-hydroxyglutarate dehydrogenase participates in the catabolism of lysine especially during senescence.The Journal of biological chemistry · 2011Article
- Isocitrate dehydrogenase 1 and 2 mutations in cancer: alterations at a crossroads of cellular metabolism.Journal of the National Cancer Institute · 2010Review
- Development and implementation of a novel assay for L-2-hydroxyglutarate dehydrogenase (L-2-HGDH) in cell lysates: L-2-HGDH deficiency in 15 patients with L-2-hydroxyglutaric aciduria.Journal of inherited metabolic disease · 2009Article
- Sudden unexpected death in an infant with L-2-hydroxyglutaric aciduria.European journal of pediatrics · 2009Review
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Authors and funding
3 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Employing lymphoblasts derived from two non related patients with L-2-HG aciduria, we examined the origin of L-2-hydroxyglutaric acid (L-2-HG) through incubation with [(13)C6]glucose and [(2)H5]glutamic acid. Formation of labelled 2-ketoglutaric acid (2-KG), citric acid and L-2-HG was determined by GC-MS. The quantitative and qualitative isotopomer pattern following incubation with [(13)C6]glucose was identical for all end-products. Incubations with [(2)H5]glutamic acid as precursor revealed the formation of identical isotopomers for 2-KG and L-2-HG. Our data indicate that 2-KG is the metabolic precursor of L-2-HG, adding to previous studies which revealed that 2-KG is the metabolic precursor of D-2-HG. These data suggest that 2-KG has a pathophysiological role in combined D/L-2-HG aciduria.
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