COL4A3 Antilichaam - middengebied: Biotine (ARP60006 P050-Biotine)

Collagen, type IV, alpha 3 (Goodpasture antigen)

ATS2, ATS3

1285

E7ENN2

NP_112730

Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Pig, Rabbit

The immunogen is a synthetic peptide directed towards the middle region of human COL4A3

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.

VHL; UBC; SERPINE2; OSM; MFAP2; FBLN2; FN1; DCN; CD93; MMP9; APP; SAA1; MATN2; TGFBI; CAMK2B; USH2A; ANTXR2; COL4A3BP; HABP2

Biotin

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

Cow: 93%; Dog: 91%; Guinea Pig: 86%; Horse: 93%; Human: 100%; Mouse: 91%; Pig: 93%; Rabbit: 85%; Rat: 91%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

156kDa

Wet Ice

1637

COL4A3

COL4A3

Rabbit

NM_031362

DPGQPGPPGEQGPPGRCIEGPRGAQGLPGLNGLKGQQGRRGKTGPKGDPG