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猪Ⅳ型胶原α3(COL4a3)酶联免疫吸附试剂盒
Catalog #: E07C1911
Sample Type: Biological samples
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Other Names

Porcine Collagen Alpha 3(IV) chain  ELISA kit

COL4A3；COL4-A3; Tumstatin; Goodpasture Antigen; Collagen Alpha-3(IV)chain

Research Area

Signal transduction;  Immunology;  Cancer

Background

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 Goodpasture's 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.