Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eleven female patients with adult-onset Still's disease were followed for 7-36 years (mean 20.2 years) after the onset of their illness. Ten of these patients had a chronic course characterized by remissions and exacerbations of arthritis associated with fever and rash. Five patients had terminal interphalangeal involvement, and carpal ankylosis was demonstrated on x-ray film in 10. Two patients developed a widespread polyarthritis, and renal amyloidosis was diagnosed 10 years after disease onset in the most severely affected patient. In 4 patients studied during an exacerbation of the disease, circulating immune complexes were detected by the staphylococcal A binding assay, but not by the C1q binding assay. Synovial fluid analysis in 1 patient revealed a low C3 level and total hemolytic complement (CH50) together with immune complexes and IgG rheumatoid factor. Immune complexes were not identified in the characteristic Still's rash by immunofluorescence or electron microscopy, although mast cell degranulation, neutrophil lysis, and perivascular fibrin deposition were reminiscent of immune complex--mediated vascular injury. The clinical and laboratory features as well as the long-term course of adult- and juvenile-onset systemic Still's disease are similar, but further studies of genetic markers and immunopathology are required to establish a common pathophysiology.
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PMID:Adult-onset Still's disease. Twenty-year followup and further studies of patients with active disease. 709 64

It is no surprise to anyone who has tried to memorize clotting and complement cascades that there are a great many trypsin-like proteases-too many, it may seem. This fecund enzyme family encompasses such a range of biological roles that other important members and functions are sure to await discovery. Of known trypsin-like proteases, few, if any, are lung-specific. Nonetheless, many contribute in ways critical to lung function, such as fighting microbial invaders, regulating the formation and removal of polymerized fibrin, and rejecting tumors or transplanted tissues. Some of these enzymes, such as plasminogen activators and mast cell tryptases, are native to the lung and live where they work; identical enzymes live and work in other tissues. Other enzymes, such as most complement and hemostatic proteases, are migrants, typically born elsewhere (e.g., the liver). They pass through the lung, looking for something to do; if nothing is found, they pass on, circulating, perhaps to return later. Still others are unwanted, uninvited intruders, such as dust mite proteases. This minireview provides a selective glimpse of the lives of some of the trypsin-like enzymes at work in the lung and airways.
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PMID:Of mites and men: trypsin-like proteases in the lungs. 919 63