Home > Product > Antibody > Rabbit Anti-PR3/FITC Conjugated antibody
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;
Cat:
SL13711R-FITC
Species Reactivity:
(predicted: Human,)
Immunogen:
KLH conjugated synthetic peptide derived from human PR3
Format:
Lyophilized or Liquid
Storage instructions:
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 ant
Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Calculated MW:
28kDa
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Unit:
Price: $
Product PDFs
Datasheet:


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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