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Serum tryptase and the laboratory diagnosis of systemic mastocytosis
Author(s): Schwartz LB, Irani AMA
Source: HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA    Volume: 14    Issue: 3    Pages: 641-+    Published: JUN 2000  
Times Cited: 24     References: 100     
Abstract: Trypsinlike activity was first associated with human mast cells by histoenzymatic stains.(10, 25, 28) Abundant and releasable trypsinlike activity was found in human lung-derived mast cells in 1981.(82) This discovery was followed by purification to homogeneity of the enzyme accounting for more than 90% of this activity; this enzyme was named tryptase.(81) The enzyme was found to be a tetramer that spontaneously and irreversibly reverted to inactive monomers at neutral pH in a physiologic salt solution unless stabilized by heparin or dextran sulfate.(2,78) In 1998, the crystal structure of lung-derived tryptase was determined,(69) confirming the tetrameric structure and the length of the heparin-binding groove previously predicted.(2) Two heparin grooves were found in each tetramer, each groove spanning the two adjacent subunits bound to one another only through weak hydrophobic interactions. All the active sites faced into the small, central pore of the planar tetramer, thereby restricting inhibitor (and substrate) access.' Because tryptase is selectively concentrated in mast cell secretory granules, it has also been studied as a clinical marker of mast cell-mediated diseases. This article reviews the molecular and biochemical biology of the human mast cell tryptase gene family and then considers the use of tryptase as a marker for systemic mastocytosis and systemic anaphylaxis.
Document Type: Article
Language: English
Reprint Address: Schwartz, LB (reprint author), Virginia Commonwealth Univ, Div Rheumatol Allergy & Immunol, McGuire Hall,Room 4-110,1112 Clay St, Richmond, VA 23219 USA
Addresses:
1. Virginia Commonwealth Univ, Div Rheumatol Allergy & Immunol, Richmond, VA 23219 USA
Publisher: W B SAUNDERS CO, INDEPENDENCE SQUARE WEST CURTIS CENTER, STE 300, PHILADELPHIA, PA 19106-3399 USA
Subject Category: Oncology; Hematology
IDS Number: 333BA
ISSN: 0889-8588
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