ReviewJournal of inherited metabolic disease2013
Metabolite proofreading, a neglected aspect of intermediary metabolism.
Review in Journal of inherited metabolic disease, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
36 citing papers in PubMed, 71 citations in OpenAlex.
- The return of metabolism: biochemistry and physiology of glycolysis.Biological reviews of the Cambridge Philosophical Society · 2026Review
- High-throughput Saccharomyces cerevisiae cultivation method for credentialing-based untargeted metabolomics.Analytical and bioanalytical chemistry · 2023Article
- Clinical and biochemical distinctions for a metabolite repair disorder caused by NAXD or NAXE deficiency.Journal of inherited metabolic disease · 2022Review
- Article
- Human cytosolic transaminases: side activities and patterns of discrimination towards physiologically available alternative substrates.Cellular and molecular life sciences : CMLS · 2022Article
- Proteomic Analysis of Mouse Kidney Tissue Associates Peroxisomal Dysfunction with Early Diabetic Kidney Disease.Biomedicines · 2022Article
- Approaches for completing metabolic networks through metabolite damage and repair discovery.Current opinion in systems biology · 2021Review
- Hypothesis: A Novel Neuroprotective Role for Glucose-6-phosphatase (G6PC3) in Brain-To Maintain Energy-Dependent Functions Including Cognitive Processes.Neurochemical research · 2020Review
- Article
- Phosphoglycolate phosphatase is a metabolic proofreading enzyme essential for cellular function inThe Journal of biological chemistry · 2019Article
- Failure to eliminate a phosphorylated glucose analog leads to neutropenia in patients with G6PT and G6PC3 deficiency.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- Role of the malonyl-CoA synthetase ACSF3 in mitochondrial metabolism.Advances in biological regulation · 2019Review
- Review
- Silencing ofOncology letters · 2018Article
- Confronting the catalytic dark matter encoded by sequenced genomes.Nucleic acids research · 2017Article
- Predicting novel substrates for enzymes with minimal experimental effort with active learning.Metabolic engineering · 2017Article
- Coupling between d-3-phosphoglycerate dehydrogenase and d-2-hydroxyglutarate dehydrogenase drives bacterial l-serine synthesis.Proceedings of the National Academy of Sciences of the United States of America · 2017Article
- A molecular rheostat maintains ATP levels to drive a synthetic biochemistry system.Nature chemical biology · 2017Article
- Evidence for Pipecolate Oxidase in Mediating Protection Against Hydrogen Peroxide Stress.Journal of cellular biochemistry · 2017Article
- The Mammalian Malonyl-CoA Synthetase ACSF3 Is Required for Mitochondrial Protein Malonylation and Metabolic Efficiency.Cell chemical biology · 2017Article
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Enzymes of intermediary metabolism are less specific than what is usually assumed: they often act on metabolites that are not their 'true' substrate, making abnormal metabolites that may be deleterious if they accumulate. Some of these abnormal metabolites are reconverted to normal metabolites by repair enzymes, which play therefore a role akin to the proofreading activities of DNA polymerases and aminoacyl-tRNA synthetases. An illustrative example of such repair enzymes is L-2-hydroxyglutarate dehydrogenase, which eliminates a metabolite abnormally made by a Krebs cycle enzyme. Mutations in L-2-hydroxyglutarate dehydrogenase lead to L-2-hydroxyglutaric aciduria, a leukoencephalopathy. Other examples are the epimerase and the ATP-dependent dehydratase that repair hydrated forms of NADH and NADPH; ethylmalonyl-CoA decarboxylase, which eliminates an abnormal metabolite formed by acetyl-CoA carboxylase, an enzyme of fatty acid synthesis; L-pipecolate oxidase, which repairs a metabolite formed by a side activity of an enzyme of L-proline biosynthesis. Metabolite proofreading enzymes are likely quite common, but most of them are still unidentified. A defect in these enzymes may account for new metabolic disorders.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.