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Rat Anti-COL6A1 Recombinant Antibody (CBCNC-280) (CBMAB-C2645-CN)


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Summary

Host Animal
Rat
Specificity
Mouse
Clone
CBCNC-280
Antibody Isotype
IgG2a
Application
IF, IHC, FC

Basic Information

Immunogen
Isolated C3H thymic stromal cells.
Host Species
Rat
Specificity
Mouse
Antibody Isotype
IgG2a
Clonality
Monoclonal Antibody
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
IHC1:50-1:100

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

Format
Liquid
Buffer
PBS
Preservative
0.09% sodium azide
Concentration
1 mg/ml

Target

Full Name
Collagen Type VI Alpha 1 Chain
Introduction
COL6A1 (Collagen Type VI Alpha 1 Chain) is a Protein Coding gene. Diseases associated with COL6A1 include Bethlem Myopathy 1 and Ullrich Congenital Muscular Dystrophy 1. Among its related pathways are Integrin Pathway and ERK Signaling. Gene Ontology (GO) annotations related to this gene include platelet-derived growth factor binding. An important paralog of this gene is COL6A2.
Entrez Gene ID
12833
UniProt ID
Q04857
Alternative Names
Collagen Type VI Alpha 1 Chain; Collagen, Type VI, Alpha 1; Collagen VI, Alpha-1 Polypeptide; Alpha 1 (VI) Chain (61 AA); Collagen Alpha-1(VI) Chain; BTHLM1; UCHMD1; 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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