ArticleThe Journal of biological chemistry2016
Developmental Defects of Caenorhabditis elegans Lacking Branched-chain α-Ketoacid Dehydrogenase Are Mainly Caused by Monomethyl Branched-chain Fatty Acid Deficiency.
Article in The Journal of biological chemistry, 2016. 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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Who cites it
21 citing papers in PubMed, 34 citations in OpenAlex.
- Article
- Dysfunctional BCAA degradation triggers neuronal damage through disrupted AMPK-mitochondrial axis due to enhanced PP2Ac interaction.Communications biology · 2025Article
- Diet and Nutrients in Rare Neurological Disorders: Biological, Biochemical, and Pathophysiological Evidence.Nutrients · 2024Review
- Quantifying acyl-chain diversity in isobaric compound lipids containing monomethyl branched-chain fatty acids.bioRxiv : the preprint server for biology · 2024Article
- An update on the secretory functions of brown, white, and beige adipose tissue: Towards therapeutic applications.Reviews in endocrine & metabolic disorders · 2024Review
- Evolutionarily related host and microbial pathways regulate fat desaturation in C. elegans.Nature communications · 2024Article
- Antioxidants, Hormetic Nutrition, and Autism.Current neuropharmacology · 2024Review
- Fasting and non-fasting plasma levels of monomethyl branched chain fatty acids: Implications for maple syrup urine disease.JIMD reports · 2023Article
- Systems-level transcriptional regulation of Caenorhabditis elegans metabolism.Molecular systems biology · 2023Article
- Dihydrolipoamide dehydrogenase, pyruvate oxidation, and acetylation-dependent mechanisms intersecting drug iatrogenesis.Cellular and molecular life sciences : CMLS · 2021Review
- Physiological effects of inactivation and the roles of Elovl3/ELOVL3 in maintaining ocular homeostasis.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2021Article
- DefiningThe Journal of biological chemistry · 2020Article
- Regulation of Nucleotide Metabolism and Germline Proliferation in Response to Nucleotide Imbalance and Genotoxic Stresses by EndoU Nuclease.Cell reports · 2020Article
- Effects of excess sugars and lipids on the growth and development ofGenes & nutrition · 2020Article
- Natural variation ineLife · 2019Article
- The nutritional requirements ofGenes & nutrition · 2019Review
- Gabapentin Can Suppress Cell Proliferation Independent of the Cytosolic Branched-Chain Amino Acid Transferase 1 (BCAT1).Biochemistry · 2018Article
- Profiling the Metabolism of Human Cells by DeepCell chemical biology · 2018Article
- Lipid and Carbohydrate Metabolism inGenetics · 2017Review
- Meibomian glands, meibum, and meibogenesis.Experimental eye research · 2017Review
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
Branched-chain α-ketoacid dehydrogenase (BCKDH) catalyzes the critical step in the branched-chain amino acid (BCAA) catabolic pathway and has been the focus of extensive studies. Mutations in the complex disrupt many fundamental metabolic pathways and cause multiple human diseases including maple syrup urine disease (MSUD), autism, and other related neurological disorders. BCKDH may also be required for the synthesis of monomethyl branched-chain fatty acids (mmBCFAs) from BCAAs. The pathology of MSUD has been attributed mainly to BCAA accumulation, but the role of mmBCFA has not been evaluated. Here we show that disrupting BCKDH in Caenorhabditis elegans causes mmBCFA deficiency, in addition to BCAA accumulation. Worms with deficiency in BCKDH function manifest larval arrest and embryonic lethal phenotypes, and mmBCFA supplementation suppressed both without correcting BCAA levels. The majority of developmental defects caused by BCKDH deficiency may thus be attributed to lacking mmBCFAs in worms. Tissue-specific analysis shows that restoration of BCKDH function in multiple tissues can rescue the defects, but is especially effective in neurons. Taken together, we conclude that mmBCFA deficiency is largely responsible for the developmental defects in the worm and conceivably might also be a critical contributor to the pathology of human MSUD.
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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.