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478356

COL6A1 (Collagen Alpha-1(VI) Chain, Collagen, Type VI, Alpha 1)

Size

£614.00

SKU:
478356
Application:
WB
Purity:
Purified by affinity chromatography.
Storage Conditions:
-20°C
Supplier:
United States Biological
Host:
Rabbit
Immunogen:
Synthetic peptide corresponding to the middle region of human COL6A1
Format:
Supplied as a liquid in PBS, 2% sucrose, 0.09% sodium azide.
Extra Details:
COL6A1 is a collagen VI which acts as a cell-binding protein.Defects in COL6A1 are a cause of Bethlem myopathy (BM).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. This gene, which encodes a neural small non-messenger RNA, is a member of the family of interspersed repetitive DNA, and its product represents an example of a primate tissue-specific RNA polymerase III transcript. The RNA sequence is divided into three domains: a 5' portion homologous to the Alu Lm, a central adenosine-rich region, and the terminal 43-nt nonrepetitive domain. It is believed that this gene was retropositionally generated and recruited into a function regulating dendritic protein biosynthesis. At least two pseudogenes of this gene have been identified. PRIMARYREFSEQ_SPAN PRIMARY_IDENTIFIER PRIMARY_SPAN COMP 1-200 AF020057.2 4262-4461 Applications: Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Shipping Conditions:
Blue Ice