CABC1 抗体 (687): sc-100663

CABC1 Antibody (687) is an IgM mouse monoclonal CABC1 antibody (also designated CABC1 antibody) that detects the CABC1 protein of human origin by WB, IP and ELISA. CABC1 Antibody (687) is available as the non-conjugated anti-CABC1 antibody. CABC1 (chaperone activity of bc1 complex-like), also known as COQ8 or ADCK3 (aarF domain-containing protein kinase 3) is a 647 amino acid mitochondrial protein that belongs to the protein kinase superfamily. Ubiquitously expressed with higher expression in heart and skeletal muscle, CABC1 is thought to function as a chaperone in the proper assembly of protein complexes found in the respiratory chain. CABC1 expression is induced both in response to DNA damage and by the tumor suppressor p53. When CABC1 expression is inhibited, p53-induced apoptosis is partially suppressed, suggesting a possible role for CABC1 in tumor suppression. Mutations in the gene encoding CABC1 may be implicated in ubiquinone deficiency which can lead to cerebellar ataxia and seizures. Four isoforms of CABC1 exist due to alternative splicing events.

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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