白细胞蛋白酶3重组兔单抗

bsm-43164R

重组兔单抗

PR3 Recombinant Rabbit mAb

白细胞蛋白酶3重组兔单抗

ACPA; AGP7; C-ANCA; CANCA; MBN; MBT; NP-4; NP4; P29; PR-3; PR3; PRTN3_HUMAN; PRTN3; C-ANCA antigen; Leukocyte proteinase 3 (PR-3 | PR3); Neutrophil proteinase 4 (NP-4); Wegener autoantigen; 3.4.21.76;

Rabbit

Recombinant human PR3 Protein

IgG

Liquid

affinity purified by Protein A

Recombinant

1A4

28 kDa

1mg/ml

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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.