Increased glutarate production by blocking the glutaryl-CoA dehydrogenation pathway and a catabolic pathway involving l-2-hydroxyglutarate
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منابع مشابه
Specific glutaryl-CoA dehydrogenating activity is deficient in cultured fibroblasts from glutaric aciduria patients.
Patients with glutaric aciduria (GA) have greatly increased urinary excretion of glutarate. Their leukocyte and fibroblast sonicates have deficient ability to produce 14CO2 from [1,5-14C]glutaryl-CoA, an enzymatic process with two sequential reaction steps, dehydrogenation and decarboxylation. In normal individuals, it is not known whether these two reaction steps require one or two enzymes, an...
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In the recent study by Wang et al. (1), the prognostic significance of the assessment of serum 2-hydroxyglutarate (2-HG) concentrations in acute myeloid leukemia was investigated. The authors report that in 87% of the patients with high serum concentrations of 2-HG, mutations in isocitrate dehydrogenase (IDH)1/IDH2 occurred, versus 29% in the group with moderately high 2-HG concentrations. This...
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BACKGROUND L-Glucose, the enantiomer of D-glucose, was believed not to be utilized by any organisms. RESULTS An L-glucose-utilizing bacterium was isolated, and its L-glucose catabolic pathway was identified genetically and enzymatically. CONCLUSION L-Glucose was utilized via a novel pathway to pyruvate and D-glyceraldehyde 3-phosphate. SIGNIFICANCE This might lead to an understanding of h...
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Enzymatic side reactions can give rise to the formation of wasteful and toxic products that are removed by metabolite repair pathways. In this work, we identify and characterize a mitochondrial metabolic repair mechanism in Arabidopsis thaliana involving malate dehydrogenase (mMDH) and l-2-hydroxyglutarate dehydrogenase (l-2HGDH). We analyze the kinetic properties of both A. thaliana mMDH isofo...
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ژورنال
عنوان ژورنال: Nature Communications
سال: 2018
ISSN: 2041-1723
DOI: 10.1038/s41467-018-04513-0