Anti-COL6A1 Antibody Picoband® fluoro488 Vervoeging

Collagen alpha-1 (VI) chain is a protein that in humans is encoded by the COL6A1 gene. 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.

Cytokine receptor common subunit beta; GM-CSF/IL-3/IL-5 receptor common beta subunit; CD131; Csf2rb; Aic2b; Csf2rb1; Il3rb1

COL6A1

1291

P12109

Human

No cross-reactivity with other proteins.

E.coli-derived human COL6A1 recombinant protein (Position: Q20-G1028) .

Broadly expressed with higher expression in heart.

Flow Cytometry

Auditory System, Domain Families, Epigenetics and Nuclear Signaling, Hlh/Leucine Zipper, Neurogenesis, Neurology Process, Neuroscience, Nuclear, Sensory System, Signaling Pathways, Stem Cells, Transcription

Immunogen affinity purified.

Liquid

High affinity receptor for interleukin-3, interleukin-5 and granulocyte-macrophage colony-stimulating factor.

1. Ackerman, C., Locke, A. E., Feingold, E., Reshey, B., Espana, K., Thusberg, J., Mooney, S., Bean, L. J. H., Dooley, K. J., Cua, C. L., Reeves, R. H., Sherman, S. L., Maslen, C. L. An excess of deleterious variants in VEGF-A pathway genes in Down-syndrome-associated atrioventricular septal defects. Am. J. Hum. Genet. 91: 646-659, 2012. 2. Baker, N. L., Morgelin, M., Pace, R. A., Peat, R. A., Adams, N. E., Gardner, R. J. M., Rowland, L. P., Miller, G., De Jonghe, P., Ceulemans, B., Hannibal, M. C., Edwards, M., Thompson, E. M., Jacobson, R., Quinlivan, R. C. M., Aftimos, S., Kornberg, A. J., North, K. N., Bateman, J. F., Lamande, S. R. Molecular consequences of dominant Bethlem myopathy collagen VI mutations. Ann. Neurol. 62: 390-405, 2007. 3. Bonaldo, P., Braghetta, P., Zanetti, M., Piccolo, S., Volpin, D., Bressan, G. M. Collagen VI deficiency induces early onset myopathy in the mouse: an animal model for Bethlem myopathy. Hum. Molec. Genet. 7: 2135-2140, 1998.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

38160 MW

6

Colony stimulating factor 2 receptor, beta, low-affinity (granulocyte-macrophage)

Membrane. Single-pass type I membrane protein.

Cone-rod homeobox protein

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.