84-4925-80　COL4A3 (Collagen, Type IV, Alpha 3 (Goodpasture Antigen)) 100ul　478351

特徴

• Type IV collagen, the major structural component of basement membranes, is a multimeric protein composed of 3 alpha subunits.
• These subunits are encoded by 6 different genes, alpha 1 through alpha 6, each of which can form a triple helix structure with 2 other subunits to form type IV collagen.
• This gene encodes alpha 3.
• In the Goodpasture syndrome, autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli.
• The epitopes that elicit these autoantibodies are localized largely to the non-collagenous C-terminal domain of the protein.
• A specific kinase phosphorylates amino acids in this same C-terminal region and the expression of this kinase is upregulated during pathogenesis.
• There are multiple alternate transcripts that appear to be unique to this human alpha 3 gene and alternate splicing is restricted to the six exons that encode this C-terminal domain.
• This gene is also linked to an autosomal recessive form of Alport syndrome.
• The mutations contributing to this syndrome are also located within the exons that encode this C-terminal region.
• Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter.
• Some exons of this gene are interspersed with exons of an uncharacterized gene which is on the opposite strand.
• 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.