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Rabbit Anti-PR3/FITC Conjugated antibody
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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