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Proteinase 3 (cANCA) - 0.2mg

Proteinase 3 (cANCA) - 0.2mg

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Proteinase 3 (cANCA)




Uniprot ID:  P24158
mRNA RefSeq:  NM_002777
Protein RefSeq:  NP_002768
Size: 0.2mg
Source: Human Neutrophils

Product Information:

Autoantibodies staining the nuclei or the perinuclear zone of neutrophils by indirect immunofluorescence are referred to as pANCA whereas those giving a clear cytoplasmic fluorescence are referred to as cANCA. The antigen recognised by most cANCA sera has been identified as proteinase 3 (also known as myeloblastin, p29 or AGP7).

Autoantibodies to proteinase 3 are found in the sera of patients with active Wegener's granulomatosis and less often in other types of systemic vasculitis including microscopic polyangiitis, idiopathic crescentic glomerulonephritis, Churg-Strauss syndrome and polyarteritis nodosa.

Proteinase 3 (PR3) is a serine proteinase with proteolytic activity towards a range of substrates. The protein has been described as having antibiotic and growth-promoting properties but has been noted to causing emphysema when administered by tracheal insufflation.

Proteinase 3 cDNA has been cloned and sequenced, revealing the protein to be a basic 25 kDa glycoprotein of 228 amino acids.  The protein reveals a triplet of bands in the range 29-32 kDa when subjected to denaturing SDS-electrophoresis, probably caused by differing degrees of glycosylation. Although proteinase 3 shows significant homology to elastase and cathepsin G, cANCA sera do not cross react with these serine proteases.

Autoantibodies to PR3 appear to recognise conformational epitopes and have been reported to interfere with inactivation of the enzyme by α1-antitrypsin and to also inhibit proteolytic activity itself. 


PDF-logo-dl Proteinase 3 (cANCA) datasheet

Clinical Indications:

Vasculitic diseases
Wegener's Granulomatosis


PDF-logo-dl  Material Safety Data Sheet


Davies, D.J. et al. (1982) Br. Med. J. 285, 606

Wiik, A. et al. (1994) Man. Biol. Markers of Dis. (Kluwer Acad.Publ.) A9:1-14

Ludemann, J. et al. (1990) J. Exp. Med. 171, 357

Jenne, D.E. et al. (1990) Nature 346, 520

Gupta, S.K. (1990) Blood, 76, 2162

Wiik, A. (1995) Semin. Clin. Immunol. 9, 5

Kallenberg, C.G.M. et al. (1994) Kidney Int. 46, 1

Kam, C.M. et al. (1992) FEBS Lett. 297, 119

Dolman, K.M. et al. (1992) FEBS Lett. 314, 117

Campanelli, D. et al. (1990) J. Exp. Med. 172, 1709

Bories, D. et al. (1989) Cell 59, 959

Kao, R.C. et al. (1988) J. Clin. Invest. 82, 1963

Wieslander, J. & Wiik, A. (1994) Man. Biol. Markers of Dis.(Kluwer Acad. Publ.) B1:1-9

Goldschmeding, R. et al. (1989) J. Clin. Invest. 84, 1577

Nassberger, L. et al. (1993) Autoimmunity 14, 259

Bini, P. et al. (1992) J. Immunol. 149, 1409

Van den Wiel, A. et al. (1992) Clin. Exp. Immunol. 90, 409

Ludemann, J. et al. (1988) J. Immunol. Meth. 114, 167

Daha, M.R. et al. (1990) Neth. J. Med. 36, 117

Kallenberg, C.G.M. & Rasmussen, N. (1990) Neth. J. Med. 36,132

Gaskin, G. et al. (1995) J. Immunol. Meth. 180, 25

Stumann, L. & Wiik, A. (1997) J. Immunol. Meth. 206, 35

Heegaard, N.H.H. et al. (1997) Anal. Biochem. 253, 259

Hagen, E.C. et al. (1993) J. Immunol. Meth. 159,1

Hagen, E.C. et al. (1996) J. Immunol. Meth. 196, 1