ArticleCell metabolism2012
Ablation of steroid receptor coactivator-3 resembles the human CACT metabolic myopathy.
Article in Cell metabolism, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 37 citations in OpenAlex.
- Androgens as the "old age stick" in skeletal muscle.Cell communication and signaling : CCS · 2025Review
- Deficiency of Nuclear Receptor Coactivator 3 Aggravates Diabetic Kidney Disease by Impairing Podocyte Autophagy.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2024Article
- Muscle Krüppel-like factor 15 regulates lipid flux and systemic metabolic homeostasis.The Journal of clinical investigation · 2021Article
- Emerging roles of steroid receptor coactivators in stromal cell responses.The Journal of endocrinology · 2021Review
- NCOA3 identified as a new candidate to explain autosomal dominant progressive hearing loss.Human molecular genetics · 2021Article
- Dudleya brittonii extract promotes survival rate and M2-like metabolic change in porcine 3D4/31 alveolar macrophages.Asian-Australasian journal of animal sciences · 2019Article
- Article
- Review
- Metabolomics and Metabolic Diseases: Where Do We Stand?Cell metabolism · 2017Review
- Association between the blood concentrations of ammonia and carnitine/amino acid of schizophrenic patients treated with valproic acid.BioPsychoSocial medicine · 2017Article
- Research Resource: Roles for Calcium/Calmodulin-Dependent Protein Kinase Kinase 2 (CaMKK2) in Systems Metabolism.Molecular endocrinology (Baltimore, Md.) · 2016Article
- Altered Energetics of Exercise Explain Risk of Rhabdomyolysis in Very Long-Chain Acyl-CoA Dehydrogenase Deficiency.PloS one · 2016Article
- Minireview: nuclear receptor coregulators of the p160 family: insights into inflammation and metabolism.Molecular endocrinology (Baltimore, Md.) · 2015Review
- Downregulation of carnitine acyl-carnitine translocase by miRNAs 132 and 212 amplifies glucose-stimulated insulin secretion.Diabetes · 2014Article
- Transcriptional coregulators: emerging roles of SRC family of coactivators in disease pathology.Journal of molecular endocrinology · 2014Review
- Hepatic SRC-1 activity orchestrates transcriptional circuitries of amino acid pathways with potential relevance for human metabolic pathogenesis.Molecular endocrinology (Baltimore, Md.) · 2014Article
- Transcriptional coregulators: fine-tuning metabolism.Cell metabolism · 2014Review
- Steroid receptor coactivators: servants and masters for control of systems metabolism.Trends in endocrinology and metabolism: TEM · 2014Review
- Steroid receptor coactivator 1 is an integrator of glucose and NAD+/NADH homeostasis.Molecular endocrinology (Baltimore, Md.) · 2014Article
- Nuclear receptor coactivators: master regulators of human health and disease.Annual review of medicine · 2014Review
Corrections and comments
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Authors and funding
21 authors at 4 institutions in 2 countries.
Funding
Abstract
Oxidation of lipid substrates is essential for survival in fasting and other catabolic conditions, sparing glucose for the brain and other glucose-dependent tissues. Here we show Steroid Receptor Coactivator-3 (SRC-3) plays a central role in long chain fatty acid metabolism by directly regulating carnitine/acyl-carnitine translocase (CACT) gene expression. Genetic deficiency of CACT in humans is accompanied by a constellation of metabolic and toxicity phenotypes including hypoketonemia, hypoglycemia, hyperammonemia, and impaired neurologic, cardiac and skeletal muscle performance, each of which is apparent in mice lacking SRC-3 expression. Consistent with human cases of CACT deficiency, dietary rescue with short chain fatty acids drastically attenuates the clinical hallmarks of the disease in mice devoid of SRC-3. Collectively, our results position SRC-3 as a key regulator of β-oxidation. Moreover, these findings allow us to consider platform coactivators such as the SRCs as potential contributors to syndromes such as CACT deficiency, previously considered as monogenic.
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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.