Anti-Collagen VI/COL6A2 Antibody Picoband® Fluoro488 Conjugated
Product Specifications
Background
Collagen alpha-2 (VI) chain is a protein that in humans is encoded by the COL6A2 gene. This gene encodes one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The product of this gene contains several domains similar to von Willebrand Factor type A domains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in this gene are associated with Bethlem myopathy and Ullrich scleroatonic muscular dystrophy. Three transcript variants have been identified for this gene.
Synonyms
ETS-related transcription factor Elf-1; E74-like factor 1; ELF1
Gene Name
COL6A2
Gene ID
1292
UniProt
P12110
Host
Rabbit
Reactivity
Human
Cross Reactivity
No cross-reactivity with other proteins.
Immunogen
E.coli-derived human Collagen VI/COL6A2 recombinant protein (Position: N35-D949) .
Clonality
Polyclonal
Tissue Specificity
In fetal tissues, it is highly expressed in heart, lung liver and kidney, and weakly expressed in brain. In adult, it is highly expressed in pancreas, spleen, thymus and peripheral blood leukocytes, expressed at moderate levels in heart, placenta, lung, liver, skeletal muscle, kidney, prostate, ovary, small intestine and colon, and weakly expressed in brain and testis.
Applications
Flow Cytometry
Field of Research
Domain Families, Epigenetics and Nuclear Signaling, Transcription
Purification
Immunogen affinity purified.
Form
Liquid
Function
Transcription factor that activates the LYN and BLK promoters. Appears to be required for the T-cell-receptor-mediated trans activation of HIV-2 gene expression. Binds specifically to two purine-rich motifs in the HIV-2 enhancer.
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. Baker, N. L., Morgelin, M., Peat, R., Goemans, N., North, K. N., Bateman, J. F., Lamande, S. R. Dominant collagen VI mutations are a common cause of Ullrich congenital muscular dystrophy. Hum. Molec. Genet. 14: 279-293, 2005.
Storage Conditions
At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
Applications Notes
6
Gene Name Synonym
E74 like ETS transcription factor 1
Subcellular Location
Nucleus.
Isotype
Rabbit IgG
Contents
Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.
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