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Mouse Anti-COL6A1 Recombinant Antibody (5C6) (CBMAB-0246-CN)


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Summary

Host Animal
Mouse
Specificity
Human, Rabbit
Clone
5C6
Antibody Isotype
IgG1
Application
ELISA

Basic Information

Specificity
Human, Rabbit
Antibody Isotype
IgG1
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!]

Format
Liquid
Purity
>95%, as determined by SDS-PAGE analysis
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Collagen Type VI Alpha 1 Chain
Introduction
The collagens are a superfamily of proteins that play a role in maintaining the integrity of various tissues. Collagens are extracellular matrix proteins and have a triple-helical domain as their common structural element. Collagen VI is a major structural component of microfibrils. The basic structural unit of collagen VI is a heterotrimer of the alpha1(VI), alpha2(VI), and alpha3(VI) chains. The alpha2(VI) and alpha3(VI) chains are encoded by the COL6A2 and COL6A3 genes, respectively. The protein encoded by this gene is the alpha 1 subunit of type VI collagen (alpha1(VI) chain). Mutations in the genes that code for the collagen VI subunits result in the autosomal dominant disorder, Bethlem myopathy.
Entrez Gene ID
Human1291
Rabbit100343947
UniProt ID
HumanP12109
Alternative Names
Collagen, Type VI, Alpha 1; Collagen VI, Alpha-1 Polypeptide; Alpha 1 (VI) Chain (61 AA); OPLL
Function
Collagen VI acts as a cell-binding protein.
Biological Process
Cell adhesion Source: UniProtKB-KW
Cellular response to amino acid stimulus Source: Ensembl
Collagen fibril organization Source: Reactome
Endodermal cell differentiation Source: UniProtKB
Extracellular matrix organization Source: Reactome
Osteoblast differentiation Source: UniProtKB
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.
PTM
Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
More Infomation

Brands, J., Steffen, F., Spennes, J., Leeb, T., & Bilzer, T. (2021). COL6A1 related muscular dystrophy in Landseer dogs: A canine model for Ullrich congenital muscular dystrophy. Muscle & nerve, 63(4), 608-616.

Zhang, Y., Liu, Z., Yang, X., Lu, W., Chen, Y., Lin, Y., ... & Yun, J. P. (2021). H3K27 acetylation activated-COL6A1 promotes osteosarcoma lung metastasis by repressing STAT1 and activating pulmonary cancer-associated fibroblasts. Theranostics, 11(3), 1473.

Li, J. Y., Liu, S. Z., Zheng, D. F., Zhang, Y. S., & Yu, M. (2021). Collagen VI-related myopathy with scoliosis alone: A case report and literature review. World Journal of Clinical Cases, 9(19), 5302.

Sato, T., Tokunaka, K., Saiga, K., Tomura, A., Sugihara, H., Hayashi, T., ... & Morita, M. (2020). Involvement of non‑triple helical type VI collagen α1 chain, NTH α1 (VI), in the proliferation of cancer cells. Oncology Reports, 44(5), 2297-2305.

Aguti, S., Bolduc, V., Ala, P., Turmaine, M., Bönnemann, C. G., Muntoni, F., & Zhou, H. (2020). Exon-skipping oligonucleotides restore functional collagen vi by correcting a common COL6A1 mutation in Ullrich CMD. Molecular Therapy-Nucleic Acids, 21, 205-216.

Yuri, P., Lestari, R. P., Fardilla, F. P., Setyaningsih, W. A. W., Arfian, N., & Dachlan, I. (2020). The impact of COL1A1 and COL6A1 expression on hypospadias and penile curvature severity. BMC urology, 20(1), 1-5.

Sato, T., Takano, R., Takahara, N., Tokunaka, K., Saiga, K., Tomura, A., ... & Morita, M. (2019). Identification of a common epitope in the sequences of COL4A1 and COL6A1 recognized by monoclonal antibody# 141. The Journal of Biochemistry, 165(1), 85-95.

Sato, T., Takano, R., Tokunaka, K., Saiga, K., Tomura, A., Sugihara, H., ... & Morita, M. (2018). Type VI collagen α1 chain polypeptide in non-triple helical form is an alternative gene product of COL6A1. The Journal of Biochemistry, 164(2), 173-181.

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

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