COL6A1 (E9U3B) Rabbit mAb #52395
- WB
- IHC
- IF
Supporting Data
| REACTIVITY | H M R |
| SENSITIVITY | Endogenous |
| MW (kDa) | 130 |
| Source/Isotype | Rabbit IgG |
Application Key:
- WB-Western Blotting
- IHC-Immunohistochemistry
- IF-Immunofluorescence
Species Cross-Reactivity Key:
- H-Human
- M-Mouse
- R-Rat
Product Information
Product Usage Information
| Application | Dilution |
|---|---|
| Western Blotting | 1:1000 |
| Immunohistochemistry (Paraffin) | 1:50 - 1:200 |
| Immunofluorescence (Frozen) | 1:400 - 1:1600 |
Storage
For a carrier free (BSA and azide free) version of this product see product #66606.
Protocol
Specificity / Sensitivity
COL6A1 (E9U3B) Rabbit mAb recognizes endogenous levels of total COL6A1 protein. This antibody is not recommended for immunohistochemical analysis of mouse tissues. Reactivity for immunofluorescence is mouse only.
Species Reactivity:
Human, Mouse, Rat
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding His1018 of human COL6A1 protein.
Background
Collagen type VI (ColVI) is a microfibrillar collagen found in the extracellular matrix (ECM) of muscles, bone, and connective tissues (1). ColVI consists of three main alpha chains: alpha 1, alpha 2, and alpha 3, which are encoded by the COL6A1, COL6A2, and COL6A3 genes, respectively. The three alpha chains form triple helix monomers and tetramers, which further assemble into a beaded microfilament network structure in the ECM (1,2). ColVI interacts with other ECM components, such as collagens, fibronectins, and perlecan, to support ECM mechanical sensing, anchoring the basement membrane to the surrounding ECM and inhibiting apoptosis and oxidative damage (3,4). Mutations in each of the ColVI genes (COL6A1, COL6A2, and COL6A3) result in defective ColVI assembly, causing Ullrich congenital muscular dystrophy (CMD) and Bethlem myopathy due to malformation of ECM structure (5,6). Knockout of COL6A1 in mice displays a mild myopathy and neurodegeneration associated with mitochondrial dysfunction, defective autophagy, and spontaneous apoptosis of muscle fibers (7,8). Increased COL6A1 in the ECM promotes tumor growth, metastasis, and therapeutic drug resistance (9-11).
- Di Martino, A. et al. (2023) Int J Mol Sci 24, 5095. doi: 10.3390/ijms24065095.
- Cescon, M. et al. (2015) J Cell Sci 128, 3525-31.
- Knupp, C. et al. (2006) J Struct Biol 154, 312-26.
- Lamandé, S.R. and Bateman, J.F. (2018) Matrix Biol 71-72, 348-367.
- Pepe, G. et al. (2006) Ann Neurol 59, 190-5.
- Bönnemann, C.G. (2011) Nat Rev Neurol 7, 379-90.
- Cescon, M. et al. (2016) Aging (Albany NY) 8, 1083-101.
- Irwin, W.A. et al. (2003) Nat Genet 35, 367-71.
- Owusu-Ansah, K.G. et al. (2019) Int J Oncol 55, 391-404.
- Zhu, Y.P. et al. (2015) Oncotarget 6, 14488-96.
- Cescon, M. et al. (2023) Cell Mol Life Sci 80, 233.
Limited Uses
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