PRTN3 Rabbit Polyclonal Antibody (ER1706-25)

UNIPROT #

P24158

PROTEIN NAME

PRTN3

SYNONYMS

ACPA antibody;AGP 7 antibody;AGP7 antibody;AGP7 serine proteinase antibody;Azurophil Granule Protein 7 antibody;C ANCA antibody;C ANCA antigen antibody;C-ANCA antigen antibody;CANCA antibody;EC 3.4.21.76 antibody;Leukocyte proteinase 3 antibody;MBN antibody;MBT antibody;MBT WEGENER AUTOANTIGEN antibody;Myeloblastin antibody;Neutrophil proteinase 4 antibody;NP 4 antibody;NP-4 antibody;NP4 antibody;P29 antibody;PR 3 antibody;PR-3 antibody;PR3 antibody;Proteinase 3 antibody;Proteinase3 antibody;PRTN 3 antibody;Prtn3 antibody;PRTN3_HUMAN antibody;Serine proteinase neutrophil Wegener granulomatosis autoantigen antibody;Serine proteinase, neutrophil antibody;Wegener autoantigen antibody;Wegener granulomatosis autoantigen antibody

SEQUENCE SIMILARITIES

Belongs to the peptidase S1 family. Elastase subfamily.

TISSUE SPECIFICITY

Expressed in polymorphonuclear leukocytes (at protein level). Expressed in neutrophils (at protein level). Expressed in differentiating neutrophils.

SUBCELLULAR LOCATION

Cytoplasmic granule, Secreted, Cell membrane, Membrane raft.

FUNCTION

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.