mHMGCS Antibody (G-11): sc-376092

mHMGCS Antibody (G-11) is a mouse monoclonal IgG1 antibody that detects mHMGCS in mouse, rat, and human samples through various applications including western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry with paraffin-embedded sections (IHCP), and enzyme-linked immunosorbent assay (ELISA). mHMGCS, or mitochondrial HMG-CoA synthase, plays a crucial role in ketone body biosynthesis by catalyzing the condensation of acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is subsequently converted into mevalonate by HMG-CoA reductase, initiating the isoprenoid biosynthesis pathway. This pathway is vital for the production of essential biomolecules such as cholesterol, ubiquinone, and dolichol, which are critical for cellular membrane integrity and energy metabolism. mHMGCS expression is primarily localized in the liver and kidney, and mHMGCS transcription is regulated by various factors including fasting, cAMP, and fatty acids, which enhance expression, while feeding and insulin exert a repressive effect. A regulatory element within the mHMGCS promoter allows for transcriptional regulation by nuclear receptors such as PPAR, RXR, COUP-TF, and HNF-4, highlighting the intricate control of this enzyme in metabolic processes. Anti-mHMGCS antibody (G-11) serves as an essential tool for researchers studying metabolic pathways and the regulation of lipid synthesis.

Alexa Fluor^® is a trademark of Molecular Probes Inc., OR., USA

LI-COR^® and Odyssey^® are registered trademarks of LI-COR Biosciences

mHMGCS Antibody (G-11) References:

1. Genetic basis of mitochondrial HMG-CoA synthase deficiency.  |  Aledo, R., et al. 2001. Hum Genet. 109: 19-23. PMID: 11479731
2. The diagnosis of mitochondrial HMG-CoA synthase deficiency.  |  Zschocke, J., et al. 2002. J Pediatr. 140: 778-80. PMID: 12072887
3. Histone deacetylase inhibitors stimulate mitochondrial HMG-CoA synthase gene expression via a promoter proximal Sp1 site.  |  Camarero, N., et al. 2003. Nucleic Acids Res. 31: 1693-703. PMID: 12626711
4. Mitochondrial HMG-CoA synthase partially contributes to antioxidant protection in the kidney of stroke-prone spontaneously hypertensive rats.  |  Yi, W., et al. 2010. Nutrition. 26: 1176-80. PMID: 20137896
5. New case of mitochondrial HMG-CoA synthase deficiency. Functional analysis of eight mutations.  |  Ramos, M., et al. 2013. Eur J Med Genet. 56: 411-5. PMID: 23751782
6. Human Mitochondrial HMG-CoA Synthase Deficiency: Role of Enzyme Dimerization Surface and Characterization of Three New Patients.  |  Puisac, B., et al. 2018. Int J Mol Sci. 19: PMID: 29597274
7. Expanding phenotypic and mutational spectra of mitochondrial HMG-CoA synthase deficiency.  |  Rojnueangnit, K., et al. 2020. Eur J Med Genet. 63: 104086. PMID: 33045405
8. Mitochondrial HMG-CoA synthase deficiency.  |  Decru, B., et al. 2025. Mol Genet Metab. 144: 109007. PMID: 39798988
9. The hepatocyte nuclear factor 4 (HNF-4) represses the mitochondrial HMG-CoA synthase gene.  |  Rodríguez, JC., et al. 1998. Biochem Biophys Res Commun. 242: 692-6. PMID: 9464279
10. Transcriptional regulation of mitochondrial HMG-CoA synthase in the control of ketogenesis.  |  Hegardt, FG. 1998. Biochimie. 80: 803-6. PMID: 9893938

The preferred mHMGCS antibody is mHMGCS Antibody (B-8): sc-393256.