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Product Name Anti-PR3/AF350 Chinese Name AF350标记的白细胞蛋白酶3抗体 Alias ACPA; AGP 7; AGP7; AGP7 serine proteinase; Azurophil Granule Protein 7; C ANCA antigen; C-ANCA antigen; EC 3.4.21.76; Leukocyte proteinase 3; MBN; MBT WEGENER AUTOANTIGEN; Myeloblastin; Neutrophil proteinase 4; NP 4; NP-4; NP4; P29; PR 3; PR-3; PR3; Proteinase3; PRTN 3; Prtn3; PRTN3_HUMAN; Serine proteinase neutrophil Wegener granulomatosis autoantigen; Wegener autoantigen. Research Area Cell biology Signal transduction lymphocyte Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, ) Applications ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 28kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human PR3 Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. Product Detail background:
The major features of Wegener granulomatosis are necrotizing granulomatous lesions, which most often affect the upper and lower airways and are associated with vasculitis, necrotizing glomerulonephritis and pulmonary capillaritis. The antigen responsible for this disease is Proteinase 3 (PR3, P29 or myeloblastin), which is one of the antibiotic proteins of neutrophilic granules belonging to the serine protease family. It is closely related to two others: Neutrophil Elastase and azurocidin. All three genes are expressed coordinately and their protein products are packaged together into azurophil granules during neutrophil differentiation. PR3 is a neutrophil protein which is able to cleave elastin and is involved in proliferation of human leukemia cells. PR3 is expressed specifically in immature myeloid cells and is a G-CSF-responsive protein critical to factor-independent growth. The genes for all three of the related serine protease family members are located in a cluster on the tip of the short arm of human chromosome 19.
Function:
Polymorphonuclear leukocyte serine protease that degrades elastin, fibronectin, laminin, vitronectin, and collagen types I, III, and IV (in vitro) and causes emphysema when administered by tracheal insufflation to hamsters.
Similarity:
Belongs to the peptidase S1 family. Elastase subfamily.
Contains 1 peptidase S1 domain.
Database links:Entrez Gene: 5657 Human
Omim: 177020 Human
SwissProt: P24158 Human
Unigene: 928 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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