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COL4A3 rabbit pAb

COL4A3 rabbit pAb

ENT-A4823

Description

 

 

 

REF ENT-A4823
Category Antibody Polyclonal
Description COL4A3 rabbit pAb
Source Rabbit
Applications IHC;IF;ELISA
Reactivity Human;Rat;Mouse;
Reactivity Human;Rat;Mouse;
Dilution Immunohistochemistry: 1/100 – 1/300. Immunofluorescence: 1/200 – 1/1000. ELISA: 1/5000. Not yet tested in other applications.
Immunogen The antiserum was produced against synthesized peptide derived from human Collagen IV alpha3. AA range:801-850
Storage Stability -20°C/1 year
Clonality Polyclonal
Isotype IgG
Concentration 1 mg/ml
Observed Band KD
Human Gene ID 1285
Human Swiss Prot Nº Q01955
Subcellular Location Secreted, extracellular space, extracellular matrix, basement membrane. Colocalizes with COL4A4 and COL4A5 in GBM, tubular basement membrane (TBM) and synaptic basal lamina (BL). .

Other Name: COL4A3; Collagen alpha-3(IV) chain; Goodpasture antigen

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 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. 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 r