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Mouse Anti-COL6A3 Recombinant Antibody (CBCNC-281) (V2LY-1206-LY699)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
CBCNC-281
Antibody Isotype
IgG1, κ
Application
WB, IP, IF, ELISA

Basic Information

Immunogen
Amino acids 301-600 mapping within an internal region of Collagen α3 Type VI of human.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:100-1:1,000
IP1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)1:50-1:500
ELISA1:100-1:1,000

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

Format
Liquid
Buffer
0.1% gelatin
Preservative
<0.1% sodium azide
Concentration
0.2 mg/ml
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Collagen Type VI Alpha 3 Chain
Entrez Gene ID
1293
UniProt ID
P12111
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.
More Infomation

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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