COL6A3 Antibody (H3-2) is a mouse monoclonal IgM antibody that detects COL6A3 in human samples through various applications including western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and immunohistochemistry. COL6A3, also known as Collagen alpha-3(VI) chain, is a 3,176 amino acid secreted protein that plays a crucial role in the structural integrity of connective tissues, particularly in the formation of the type VI collagen complex. COL6A3 is characterized by its unique structure, which includes one fibronectin type-III domain, one MPTI inhibitor domain, and twelve von Willebrand factor A (VWFA) domains, allowing COL6A3 to form a stable trimer with two other type VI alpha chains. The structural composition of COL6A3 is vital for its function as a cell-binding protein, facilitating the organization of extracellular matrix components and contributing to tissue resilience. Mutations in the COL6A3 gene are linked to severe muscle disorders such as Bethlem myopathy and Ullrich congenital muscular dystrophy, highlighting COL6A3′s significance in maintaining muscle and connective tissue health. Multiple isoforms of COL6A3 due to alternative splicing further underscore COL6A3′s functional diversity and importance in various biological processes.
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COL6A3 Antibody (H3-2) References:
- Clinical significance of serum COL6A3 in pancreatic ductal adenocarcinoma. | Kang, CY., et al. 2014. J Gastrointest Surg. 18: 7-15. PMID: 24002763
- Position of glycine substitutions in the triple helix of COL6A1, COL6A2, and COL6A3 is correlated with severity and mode of inheritance in collagen VI myopathies. | Butterfield, RJ., et al. 2013. Hum Mutat. 34: 1558-67. PMID: 24038877
- COL6A3 expression in adipocytes associates with insulin resistance and depends on PPARγ and adipocyte size. | Dankel, SN., et al. 2014. Obesity (Silver Spring). 22: 1807-13. PMID: 24719315
- COL6A3 is regulated by leptin in human adipose tissue and reduced in obesity. | McCulloch, LJ., et al. 2015. Endocrinology. 156: 134-46. PMID: 25337653
- The role of mutations in COL6A3 in isolated dystonia. | Lohmann, K., et al. 2016. J Neurol. 263: 730-4. PMID: 26872670
- COL6A3 promotes cellular malignancy of osteosarcoma by activating the PI3K/AKT pathway. | Guo, HL., et al. 2020. Rev Assoc Med Bras (1992). 66: 740-745. PMID: 32696868
- COL6A3 expression in adipose tissue cells is associated with levels of the homeobox transcription factor PRRX1. | Dankel, SN., et al. 2020. Sci Rep. 10: 20164. PMID: 33214660
- A Damaging COL6A3 Variant Alters the MIR31HG-Regulated Response of Chondrocytes in Neocartilage Organoids to Hyperphysiologic Mechanical Loading. | Bloks, NG., et al. 2024. Adv Sci (Weinh). 11: e2400720. PMID: 39021299
- COL6A3 Exosomes Promote Tumor Dissemination and Metastasis in Epithelial Ovarian Cancer. | Ho, CM., et al. 2024. Int J Mol Sci. 25: PMID: 39125689
- Single-cell and spatial transcriptomics identify COL6A3 as a prognostic biomarker in undifferentiated pleomorphic sarcoma. | Klein, JC., et al. 2024. Mol Cancer. 23: 257. PMID: 39548577