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Introduction | Type IV collagen is a major structural component of basement membranes. It is a multimeric protein composed of 3 alpha subunits, which are encoded by 6 different genes, alpha 1 throµgh alpha 6. Each of these alpha subunits can form a triple helix structure with 2 other subunits to form type IV collagen. The Goodpasture syndrome is a condition in which autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli. The epitopes that elicit these autoantibodies are restricted basically to the non-collagenous C-terminal domain of the protein. There are numerous alternative transcripts that appear to be unique to the human COL4A3 and alternative splicing is limited to the six exons that encode this C-terminal domain. COL4A3 is also linked to an autosomal recessive form of Alport syndrome. The mutations contributing to the Alport syndrome are also situated within the exons that encode this C-terminal region. COL4A3 is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter. Several exons of COL4A3 are interspersed with exons of an uncharacterized gene which is on the opposite strand. |
Synonyms | Collagen alpha-3(IV) chain, Goodpasture antigen, COL4A3, Glomerular Basal Membrane, GBM. |
Source | Sf9 insect cells. |
Physical Appearance | Sterile Filtered clear solution. |
Formulation | The protein solution (0.6 mg/ml) contains 20mM Hepes, pH 8.0, and 4M Urea |
Stability | Recommendations for storage buffer: Ionic strength between 50 and 100 mM, neutral to slightly alkaline pH and 4 M urea as dissociating agent. Storage temperature is -70 |
Usage | NeoScientific's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drµgs, agricultural or pesticidal products, food additives or household chemicals. |
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