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Secretory leukocyte protease inhibitor in cystic fibrosis. 1993

S C Stromatt
Clinical Research Department, Synergen, Boulder, CO 80301.

Cystic fibrosis (CF) is a genetic disorder that leads to a defect in chloride ion transport and results in pancreatic and pulmonary insufficiency. The pulmonary disease is characterized by bacterial colonization and inflammation with excessive levels of neutrophils and neutrophil elastase within the lung. Neutrophil elastase is considered to be one of the major mediators of the pulmonary damage. Secretory leukocyte protease inhibitor (SLPI) is a natural anti-protease found in the upper airways and has been successfully produced by recombinant technology. SLPI is effective in reducing elastase-induced damage in vitro and in vivo and has recently been administered safely as an aeroeol to CF patients with evidence of biochemical efficacy.

UI MeSH Term Description Entries
D010196 Pancreatic Elastase A protease of broad specificity, obtained from dried pancreas. Molecular weight is approximately 25,000. The enzyme breaks down elastin, the specific protein of elastic fibers, and digests other proteins such as fibrin, hemoglobin, and albumin. EC 3.4.21.36. Elastase,Pancreatopeptidase,Elastase I,Pancreatic Elastase I,Elastase I, Pancreatic,Elastase, Pancreatic
D011506 Proteins Linear POLYPEPTIDES that are synthesized on RIBOSOMES and may be further modified, crosslinked, cleaved, or assembled into complex proteins with several subunits. The specific sequence of AMINO ACIDS determines the shape the polypeptide will take, during PROTEIN FOLDING, and the function of the protein. Gene Products, Protein,Gene Proteins,Protein,Protein Gene Products,Proteins, Gene
D011994 Recombinant Proteins Proteins prepared by recombinant DNA technology. Biosynthetic Protein,Biosynthetic Proteins,DNA Recombinant Proteins,Recombinant Protein,Proteins, Biosynthetic,Proteins, Recombinant DNA,DNA Proteins, Recombinant,Protein, Biosynthetic,Protein, Recombinant,Proteins, DNA Recombinant,Proteins, Recombinant,Recombinant DNA Proteins,Recombinant Proteins, DNA
D003550 Cystic Fibrosis An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION. Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D015842 Serine Proteinase Inhibitors Exogenous or endogenous compounds which inhibit SERINE ENDOPEPTIDASES. Serine Endopeptidase Inhibitor,Serine Endopeptidase Inhibitors,Serine Protease Inhibitor,Serine Protease Inhibitors,Serine Proteinase Antagonist,Serine Proteinase Antagonists,Serine Proteinase Inhibitor,Serine Proteinase Inhibitors, Endogenous,Serine Proteinase Inhibitors, Exogenous,Serine Protease Inhibitors, Endogenous,Serine Protease Inhibitors, Exogenous,Antagonist, Serine Proteinase,Endopeptidase Inhibitor, Serine,Inhibitor, Serine Endopeptidase,Inhibitor, Serine Protease,Inhibitor, Serine Proteinase,Protease Inhibitor, Serine,Proteinase Antagonist, Serine,Proteinase Inhibitor, Serine
D053481 Secretory Leukocyte Peptidase Inhibitor A proteinase inhibitor found in various BODILY SECRETIONS that coat mucosal surfaces such as SEMINAL PLASMA; CERVICAL MUCUS; and bronchial secretions. It plays a role in protecting epithelial tissues from LEUKOCYTE-derived serine proteases such as NEUTROPHIL ELASTASE. Antileukoprotease,Bronchial Mucus Elastase Inhibitor,Elastase Inhibitor, Mucus,Leukocyte Peptidase Inhibitor, Secretory,Mucus Protease Inhibitor,Mucus Proteinase Inhibitor,Secretory Leukocyte-Protease Inhibitor,Secretory Leukoprotease Inhibitor,Seminal Proteinase Inhibitor,Mucus Elastase Inhibitor,Protease Inhibitor, Mucus,Proteinase Inhibitor, Mucus,Secretory Leukocyte Protease Inhibitor
D053491 Proteinase Inhibitory Proteins, Secretory Peptides and proteins found in BODILY SECRETIONS and BODY FLUIDS that are PROTEASE INHIBITORS. They play a role in INFLAMMATION, tissue repair and innate immunity (IMMUNITY, INNATE) by inhibiting endogenous proteinases such as those produced by LEUKOCYTES and exogenous proteases such as those produced by invading microorganisms. Neutrophil Elastase Inhibitor,Neutrophil Elastase Inhibitors,Proteinase Inhibitory Peptides, Secretory,Peptidase Inhibitory Peptides, Secretory,Peptidase Inhibitory Proteins, Secretory,Protease Inhibitory Peptides, Secretory,Protease Inhibitory Proteins, Secretory,Elastase Inhibitor, Neutrophil,Elastase Inhibitors, Neutrophil,Inhibitor, Neutrophil Elastase,Inhibitors, Neutrophil Elastase
D019272 Leukocyte Elastase An enzyme that catalyzes the hydrolysis of proteins, including elastin. It cleaves preferentially bonds at the carboxyl side of Ala and Val, with greater specificity for Ala. EC 3.4.21.37. Lysosomal Elastase,Neutrophil Elastase,PMN Elastase,Polymorphonuclear Leukocyte Elastase,Granulocyte Elastase,Elastase, Granulocyte,Elastase, Leukocyte,Elastase, Lysosomal,Elastase, Neutrophil,Elastase, PMN,Elastase, Polymorphonuclear Leukocyte,Leukocyte Elastase, Polymorphonuclear

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