P0615

Sigma-Aldrich

Proteinase 3 from human leukocytes

≥95% (PAGE)

Synonym(s):
Myeloblastin, PR3 Ag, PR-3, P29B, AGP7
Enzyme Commission number:
MDL number:
NACRES:
NA.54
Pricing and availability is not currently available.

Quality Level

assay

≥95% (PAGE)

form

solid

specific activity

≥25 units/mg protein

storage temp.

−20°C

Gene Information

human ... PRTN3(5657)

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General description

Proteinase 3 (PR3) is mapped to human chromosome 19p13.3. Proteinase 3 is present majorly in azurophilic granules and belongs to neutrophil-derived proteases family. PR3 is a peripheral membrane protein and lacks transmembrane domain. It comprises of an unique interfacial binding site (IBS) and two amino acid clusters, one charged and one hydrophobic, for membrane association.
Proteinase 3 is found in high concentration in the primary granules of myeloid leukemia blasts.

Application

Proteinase 3 has been shown to be important in the pathogenesis of human emphysema. It has also been used in a study to investigate the liberation of the antibacterial and cytotoxic peptide LL-37 by cleavage of human cathelicidin hCAP-183.

Biochem/physiol Actions

Proteinase 3 (PR-3) is a serine protease expressed in the granules of neutrophils and monocytes. Also known as myeloblastin and p29b, it degrades elastin and is associated with myeloid leukemia and Wegener′s granulomatosis.
Used for the detection of anti-PR3 auto-antibodies in immunoassays.

Unit Definition

One unit will hydrolyze 1 μmole of Boc-Ala-Ala-Nva-SBzl per minute at pH 7.5 at 25 deg C.

RIDADR

NONH for all modes of transport

WGK Germany

3

Certificate of Analysis
Certificate of Origin
Proteinase 3 is a phosphatidylserine binding protein which affects the production and function of microvesicles
Martin KR, et al.
The Journal of biological chemistry, jbc-M115 (2016)
Y M van der Geld et al.
Journal of leukocyte biology, 69(2), 177-190 (2001-03-29)
Proteinase 3 (PR3) is one of four serine protease homologues in the azurophilic granules of neutrophils and granules of monocytes. It is of importance that anti-neutrophil cytoplasmic antibodies (ANCA) in patients with Wegener's granulomatosis (WG) are mainly directed against PR3...
Genetics and pathophysiology of granulomatosis with polyangiitis (GPA) and its main autoantigen proteinase 3
Relle M, et al.
Molecular and Cellular Probes, 30(6), 366-373 (2016)
Molecular analysis of the membrane insertion domain of proteinase 3, the Wegener?s autoantigen, in RBL cells: implication for its pathogenic activity
Kantari C, et al.
Journal of Leukocyte Biology, 90(5), 941-950 (2011)
M M Boomsma et al.
Journal of immunological methods, 254(1-2), 47-58 (2001-06-19)
The prevalence of Anti-Neutrophil Cytoplasmic Antibodies (ANCA) directed against myeloperoxidase (MPO) in pauci-immune necrotizing crescentic glomerulonephritis (NCGN) is dependent on the assay(s) used. We investigated the frequency of MPO-ANCA as detected by different assays for MPO-ANCA in a large cohort...

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