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Blood, 1 April 2004, Vol. 103, No. 7, pp. 2710-2717. Prepublished online as a Blood First Edition Paper on December 11, 2003; DOI 10.1182/blood-2003-08-2981. This Article Full Text (PDF) All Versions of this Article: 2003-08-2981v1 103/7/2710    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Strik, M. C M Articles by Kummer, J. A. Search for Related Content PubMed PubMed Citation Articles by Strik, M. C M Articles by Kummer, J. A. Social Bookmarking                   What's this? Submitted August 29, 2003 Accepted November 24, 2003 The intracellular serpin SERPINB6 (PI6) is abundantly expressed by human mast cells and forms complexes with -tryptase monomers Merel C M Strik*, Angela Wolbink, Dorine Wouters, Bellinda A Bladergroen, Angelique R Verlaan, Inge S van Houdt, Sanne Hijlkema, C Erik Hack, and Jean Alain Kummer Department of Clinical Chemistry, VU University Medical Center, Amsterdam, The Netherlands; Department of Immunopathology, Sanquin Research at CLB, Amsterdam, The Netherlands Department of Pathology, VU University Medical Center, Amsterdam, The Netherlands * Corresponding author; email: m.strik{at}sanquin.nl. SERPINB6 (PI6) is member of the intracellular serine protease inhibitors (serpins). Previous studies showed that SERPINB6 is localized mainly in the cytoplasm of endothelial cells, epithelial cells, monocytes and neutrophils and in these cells SERPINB6 is supposed to prevent cellular damage by scavenging leaking lysosomal proteases. In this paper we show, using novel well-defined monoclonal antibodies, that SERPINB6 is abundantly expressed by mast cells in all organs, as well as by the human mast cell line HMC-1. Gel filtration experiments revealed that the latter cells contain a high molecular weight form of SERPINB6, which consists of SDS-stable complexes of this inhibitor with monomeric beta-tryptase. Expression of SERPINB6 by mast cells was compared to those of tryptase and CD117 (c-kit) in biopsies from patients with different forms of mast cell disease. In all cases the lesional mast cells expressed SERPINB6 and in diffuse cutaneous mastocytosis and mastocytoma SERPINB6 was expressed by a substantial higher number of mast cells when compared to tryptase. In conclusion, SERPINB6 is abundantly expressed by normal mast cells and by mast cells in mastocytoma lesions. We suggest that in mast cells SERPINB6 serves to regulate activity of endogenous beta-tryptase in the cytoplasm. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Bots and J. P. Medema Serpins in T cell immunity J. Leukoc. Biol., November 1, 2008; 84(5): 1238 - 1247. [Abstract] [Full Text] [PDF] F. L. Scott, J. Sun, J. C. Whisstock, K. Kato, and P. I. Bird SerpinB6 is an Inhibitor of Kallikrein-8 in Keratinocytes J. Biochem., October 1, 2007; 142(4): 435 - 442. [Abstract] [Full Text] [PDF] M. I. Zervou, E. G. Tzortzaki, D. Makris, M. Gaga, E. Zervas, E. Economidou, M. Tsoumakidou, N. Tzanakis, J. Milic-Emili, and N. M. Siafakas Differences in microsatellite DNA level between asthma and chronic obstructive pulmonary disease Eur. Respir. J., September 1, 2006; 28(3): 472 - 478. [Abstract] [Full Text] [PDF]

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