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Mouse Anti-COL6A1 Recombinant Antibody (CBCNC-279) (V2LY-1206-LY695)


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Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
CBCNC-279
Antibody Isotype
IgG2a, κ
Application
WB, IP, IF, ELISA

Basic Information

Immunogen
Amino acids 51-250 mapping near the N-terminus of Collagen α1 Type VI of human.
Host Species
Mouse
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG2a, 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 1 Chain
Entrez Gene ID
Human1291
Mouse12833
Rat294337
UniProt ID
HumanP12109
MouseQ04857
RatD3ZUL3
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.

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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