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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 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 Citation Map 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. Related Collections Immunobiology Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  IMMUNOBIOLOGY 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 J. Alain Kummer From the Departments of Clinical Chemistry and Pathology, VU University Medical Center, Amsterdam; and the Department of Immunopathology, Sanquin Research at CLB, and Landsteiner Laboratory, Academical Medical Centre, Amsterdam, the Netherlands. SERPINB6 (PI6) is a member of the intracellular serine protease inhibitors (serpins). Previous studies showed that SERPINB6 is localized mainly in the cytoplasm of endothelial cells, some epithelial cells, monocytes, and neutrophils. In these cells SERPINB6 is thought to prevent cellular damage by scavenging leaking lysosomal proteases. We show here, using novel, well-defined monoclonal antibodies, that SERPINB6 is abundantly expressed by mast cells in all organs and 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 sodium dodecyl sulfate (SDS)-stable complexes of this inhibitor with monomeric -tryptase. Expression of SERPINB6 by mast cells was compared with 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 substantially higher number of mast cells when compared with 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 the activity of endogenous -tryptase in the cytoplasm. (Blood. 2004;103:2710-2717) CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: 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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