ArticleThe Journal of biological chemistry2011
Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading.
Article in The Journal of biological chemistry, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers, 1 of them a synthesis that pooled it.
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Who cites it
38 citing papers in PubMed, 1 synthesis or guideline pooled it, 56 citations in OpenAlex.
- Multi-modal meta-analysis of cancer cell line omics profiles identifies ECHDC1 as a novel breast tumor suppressor.Molecular systems biology · 2021Pooled it
- Comparative proteomic analysis of skin wound healing responses to biomaterial treatments identifies key pathways which govern differential regenerative outcomes.Biomaterials · 2026Article
- Comprehensive Analysis of Immune-Related Mitochondrial Genes in Ischemic Stroke Through Integrated Bioinformatics and Validation.Biomedicines · 2026Article
- Impact of keto leucine and isoleucine on CHO cell central carbon metabolism and performance in fed-batch and steady-state perfusion.Frontiers in bioengineering and biotechnology · 2026Article
- Machine learning-based identification of targeted metabolomic biomarkers for early diagnosis and fibrosis-stage discrimination in metabolic dysfunction-associated steatotic liver disease.Frontiers in nutrition · 2026Article
- Integrated Transcriptomic and Proteomic Analysis Associated with Knockdown and Overexpression Studies Revealed ECHDC1 as a Regulator of Intramuscular Fat Deposition in Cattle.Animals : an open access journal from MDPI · 2025Article
- Integrative QTL Mapping and Transcriptomic Profiling to Identify Growth-Associated QTL and Candidate Genes in Hong Kong Catfish (Animals : an open access journal from MDPI · 2025Article
- Mining cholesterol genes from thousands of mouse livers identifies aldolase C as a regulator of cholesterol biosynthesis.Journal of lipid research · 2024Article
- Proteomics Analysis of Pregnancy in Ewes under Heat Stress Conditions and Melatonin Administration.Animals : an open access journal from MDPI · 2024Article
- Article
- High-throughput Saccharomyces cerevisiae cultivation method for credentialing-based untargeted metabolomics.Analytical and bioanalytical chemistry · 2023Article
- Polyphenols and IUGR Pregnancies: Effects of the Antioxidant Hydroxytyrosol on the Hippocampus Proteome in a Porcine Model.Antioxidants (Basel, Switzerland) · 2022Article
- Characteristics of Urine Organic Acid Metabolites in Nonalcoholic Fatty Liver Disease Assessed Using Magnetic Resonance Imaging with Elastography in Korean Adults.Diagnostics (Basel, Switzerland) · 2022Article
- Transcriptome analysis reveals the potential roles of long non-coding RNAs in feed efficiency of chicken.Scientific reports · 2022Article
- Biosynthesis of Polyhydroxyalkanoate Terpolymer from Methanol via the Reverse β-Oxidation Pathway in the Presence of Lanthanide.Microorganisms · 2022Article
- Biosynthesis of Poly(3-hydroxybutyrate-Frontiers in bioengineering and biotechnology · 2022Article
- Approaches for completing metabolic networks through metabolite damage and repair discovery.Current opinion in systems biology · 2021Review
- Variants in the ethylmalonyl-CoA decarboxylase (ECHDC1) gene: a novel player in ethylmalonic aciduria?Journal of inherited metabolic disease · 2021Article
- The human type 2 diabetes-specific visceral adipose tissue proteome and transcriptome in obesity.Scientific reports · 2021Article
- Article
Corrections and comments
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Authors and funding
8 authors at 5 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
A limited number of enzymes are known that play a role analogous to DNA proofreading by eliminating non-classical metabolites formed by side activities of enzymes of intermediary metabolism. Because few such "metabolite proofreading enzymes" are known, our purpose was to search for an enzyme able to degrade ethylmalonyl-CoA, a potentially toxic metabolite formed at a low rate from butyryl-CoA by acetyl-CoA carboxylase and propionyl-CoA carboxylase, two major enzymes of lipid metabolism. We show that mammalian tissues contain a previously unknown enzyme that decarboxylates ethylmalonyl-CoA and, at lower rates, methylmalonyl-CoA but that does not act on malonyl-CoA. Ethylmalonyl-CoA decarboxylase is particularly abundant in brown adipose tissue, liver, and kidney in mice, and is essentially cytosolic. Because Escherichia coli methylmalonyl-CoA decarboxylase belongs to the family of enoyl-CoA hydratase (ECH), we searched mammalian databases for proteins of uncharacterized function belonging to the ECH family. Combining this database search approach with sequencing data obtained on a partially purified enzyme preparation, we identified ethylmalonyl-CoA decarboxylase as ECHDC1. We confirmed this identification by showing that recombinant mouse ECHDC1 has a substantial ethylmalonyl-CoA decarboxylase activity and a lower methylmalonyl-CoA decarboxylase activity but no malonyl-CoA decarboxylase or enoyl-CoA hydratase activity. Furthermore, ECHDC1-specific siRNAs decreased the ethylmalonyl-CoA decarboxylase activity in human cells and increased the formation of ethylmalonate, most particularly in cells incubated with butyrate. These findings indicate that ethylmalonyl-CoA decarboxylase may correct a side activity of acetyl-CoA carboxylase and suggest that its mutation may be involved in the development of certain forms of ethylmalonic aciduria.
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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.