CABC1 Antibody (687): sc-100663

CABC1 Antibody (687) is a mouse monoclonal IgM antibody that detects CABC1 protein of human origin by western blotting (WB), immunoprecipitation (IP), and enzyme-linked immunosorbent assay (ELISA). Anti-CABC1 antibody (687) is available as the non-conjugated format. CABC1, also known as COQ8 or ADCK3 (aarF domain-containing protein kinase 3), is a 647 amino acid mitochondrial protein that plays a crucial role in assembling protein complexes within the respiratory chain, essential for cellular energy production. CABC1 is ubiquitously expressed, with particularly high levels in heart and skeletal muscle, highlighting its importance in tissues with high energy demands. CABC1 expression increases in response to DNA damage and is influenced by tumor suppressor p53, indicating potential involvement in cellular stress responses and tumor suppression. Inhibition of CABC1 expression partially suppresses p53-induced apoptosis, suggesting a significant role in preventing tumorigenesis. Mutations in CABC1 have been linked to ubiquinone deficiency, which can result in severe neurological disorders such as cerebellar ataxia and seizures. Four isoforms of CABC1 exist due to alternative splicing, which may contribute to diverse functional roles in cellular metabolism and stress response.

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

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

CABC1 Antibody (687) References:

1. Isolation of a novel gene, CABC1, encoding a mitochondrial protein that is highly homologous to yeast activity of bc1 complex.  |  Iiizumi, M., et al. 2002. Cancer Res. 62: 1246-50. PMID: 11888884
2. Large-scale cDNA transfection screening for genes related to cancer development and progression.  |  Wan, D., et al. 2004. Proc Natl Acad Sci U S A. 101: 15724-9. PMID: 15498874
3. CABC1 gene mutations cause ubiquinone deficiency with cerebellar ataxia and seizures.  |  Mollet, J., et al. 2008. Am J Hum Genet. 82: 623-30. PMID: 18319072
4. ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency.  |  Lagier-Tourenne, C., et al. 2008. Am J Hum Genet. 82: 661-72. PMID: 18319074