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Mouse Anti-COL6A3 Recombinant Antibody (CBCNC-281) (CBMAB-C2647-CN)

This product is a Mouse antibody that recognizes COL6A3. The antibody CBCNC-281 can be used for immunoassay techniques such as: WB, ICC, IHC-P, IHC-F, ELISA.
See all COL6A3 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBCNC-281
Application
WB, ICC, IHC-P, IHC-F, ELISA

Basic Information

Immunogen
Collagen Type VI Alpha 3
Specificity
Human
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
PBS, pH 7.4, 50% glycerol
Preservative
0.02% Sodium azide
Concentration
0.5 mg/mL

Target

Full Name
Collagen Type VI Alpha 3 Chain
Introduction
COL6A3 (Collagen Type VI Alpha 3 Chain) is a Protein Coding gene. Diseases associated with COL6A3 include Dystonia 27 and Bethlem Myopathy 1. Among its related pathways are Integrin Pathway and ERK Signaling. Gene Ontology (GO) annotations related to this gene include serine-type endopeptidase inhibitor activity. An important paralog of this gene is COL6A5.
Entrez Gene ID
1293
UniProt ID
P12111
Alternative Names
Collagen Type VI Alpha 3 Chain; Collagen, Type VI, Alpha 3; Collagen VI, Alpha-3 Polypeptide; Collagen Alpha-3(VI) Chain; BTHLM1; UCMD1; DYT27;
Function
Collagen VI acts as a cell-binding protein.
Biological Process
Cell adhesion Source: UniProtKB-KW
Collagen fibril organization Source: Reactome
Extracellular matrix organization Source: Reactome
Muscle organ development Source: ProtInc
Cellular Location
Extracellular matrix
Involvement in disease
Bethlem myopathy 1 (BTHLM1):
A benign proximal myopathy characterized by early childhood onset and joint contractures most frequently affecting the elbows and ankles.
Ullrich congenital muscular dystrophy 1 (UCMD1):
A congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy.
Dystonia 27 (DYT27):
A form of dystonia, a disorder defined by the presence of sustained involuntary muscle contraction, often leading to abnormal postures. DYT27 is an autosomal recessive form characterized by segmental isolated dystonia involving the face, neck, bulbar muscles, and upper limbs.
PTM
Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
The N-terminus is blocked.

Jin, C. Y., Zheng, R., Lin, Z. H., Xue, N. J., Chen, Y., Gao, T., ... & Zhang, B. R. (2021). Study of the collagen type VI alpha 3 (COL6A3) gene in Parkinson’s disease. BMC neurology, 21(1), 1-7.

Stavusis, J., Micule, I., Wright, N. T., Straub, V., Topf, A., Panadés-de Oliveira, L., ... & Lace, B. (2020). Collagen VI-related limb-girdle syndrome caused by frequent mutation in COL6A3 gene with conflicting reports of pathogenicity. Neuromuscular Disorders, 30(6), 483-491.

Wang, J., & Pan, W. (2020). The biological role of the collagen alpha-3 (Vi) chain and its cleaved c5 domain fragment endotrophin in cancer. OncoTargets and therapy, 13, 5779.

Holm Nielsen, S., Mortensen, J. H., Willumsen, N., Rasmussen, D. G. K., Mogensen, D. J., Di Sabatino, A., ... & Genovese, F. (2020). A fragment of collagen type VI alpha-3 chain is elevated in serum from patients with gastrointestinal disorders. Scientific Reports, 10(1), 1-8.

Duan, Y., Liu, G., Sun, Y., Wu, J., Xiong, Z., Jin, T., & Chen, M. (2019). COL6A3 polymorphisms were associated with lung cancer risk in a Chinese population. Respiratory research, 20(1), 1-8.

Peng, X. Y., Qu, Y. J., Song, F., Sun, X. F., Ge, X. S., & Jiao, H. (2019). Clinical manifestations and genetics analysis of collagen type Ⅵ-related myopathy caused by variants in COL6A3 gene. Zhonghua er ke za zhi= Chinese Journal of Pediatrics, 57(2), 136-141.

Yu, J., Zhang, B., Zhang, H., Qi, Y., Wang, Y., Wang, W., ... & Wang, Y. (2019). E2F1-induced upregulation of long non-coding RNA LMCD1-AS1 facilitates cholangiocarcinoma cell progression by regulating miR-345–5p/COL6A3 pathway. Biochemical and biophysical research communications, 512(2), 150-155.

Huang, Y., Li, G., Wang, K., Mu, Z., Xie, Q., Qu, H., ... & Hu, B. (2018). Collagen type VI alpha 3 chain promotes epithelial-mesenchymal transition in bladder cancer cells via transforming growth factor β (TGF-β)/Smad pathway. Medical science monitor: international medical journal of experimental and clinical research, 24, 5346.

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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