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Anti-COL6A1 Antibody Picoband® Fluoro594 Conjugated

Product Specifications

Background

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.

Synonyms

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

Gene Name

COL6A1

Gene ID

1291

UniProt

P12109

Host

Rabbit

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

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

Clonality

Polyclonal

Tissue Specificity

Broadly expressed with higher expression in heart.

Applications

Flow Cytometry

Field of Research

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

Purification

Immunogen affinity purified.

Form

Liquid

Function

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

References & Citations

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.

Storage Conditions

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

Calculated Molecular Weight

38160 MW

Applications Notes

6

Gene Name Synonym

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

Subcellular Location

Membrane. Single-pass type I membrane protein.

Protein Name

Cone-rod homeobox protein

Isotype

Rabbit IgG

Contents

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

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