COL4A3 Antibody - N-terminal region (ARP60007_P050)

• Product Number: ARP60007_P050
• Name: COL4A3 Antibody - N-terminal region (ARP60007_P050)
• Protein Size (# AA): 1637 amino acids
• Molecular Weight: 156kDa
• NCBI Gene Id: 1285
• Host: Rabbit
• Clonality: Polyclonal
• Concentration: 0.5 mg/ml
• Gene Full Name: Collagen, type IV, alpha 3 (Goodpasture antigen)
• Alias Symbols: ATS2, ATS3
• Peptide Sequence: Synthetic peptide located within the following region: GPPGVPGSPGSSRPGLRGAPGWPGLKGSKGERGRPGKDAMGTPGSPGCAG
• Product Format: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Description of Target: 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.
• Protein Interactions: VHL; UBC; SERPINE2; OSM; MFAP2; FBLN2; FN1; DCN; CD93; MMP9; APP; SAA1; MATN2; TGFBI; CAMK2B; USH2A; ANTXR2; COL4A3BP; HABP2;
• Reconstitution and Storage: For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
• Blocking Peptide: For anti-COL4A3 (ARP60007_P050) antibody is Catalog # AAP60007 (Previous Catalog # AAPP46160)
• Immunogen: The immunogen is a synthetic peptide directed towards the N terminal region of human COL4A3
• Uniprot ID: E7ENN2
• Protein Accession #: NP_112730
• Purification: Affinity Purified
• Nucleotide Accession #: NM_031362
• Tested Species Reactivity: Human
• Gene Symbol: COL4A3
• Predicted Species Reactivity: Human, Mouse, Guinea Pig, Horse, Rabbit
• Application: WB
• Predicted Homology Based on Immunogen Sequence: Guinea Pig: 85%; Horse: 86%; Human: 100%; Mouse: 92%; Rabbit: 85%
• Image 1: Human Liver
  
  WB Suggested Anti-COL4A3 Antibody Titration: 0.2-1 ug/ml
  ELISA Titer: 1:62500
  Positive Control: Human Liver