Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.21.4 (trypsin)
 42,187 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The present study using immunologic methodology confirms previous observations from this laboratory of an absence of a protease component with arginine esterase activity in plasma of patients with cystic fibrosis. In this study, the pooled plasma from control individuals was activated and partially purified after adsorption on columns of soybean trypsin inhibitor conjugated to Sepharose 4B followed by elution with benzamidine. The fraction was further purified by isoelectrofocusing on polyacrylamide gels. Proteins around the pI range of 5.5 were eluted and utilized to prepare an antiserum. Immunoelectrophoresis of activated plasma samples from control subjects and patients with cystic fibrosis was performed utilizing the antiserum. In controls, four precipitin arcs with residual esterase activity were observed, whereas only three were seen in plasma from patients with cystic fibrosis. Double gel diffusion experiments using specific antisera ruled out the presence of trypsin, chymotrypsin, plasminogen, prothrombin, C1 esterase, alpha one-trypsin inhibitor, and inter-alpha-trypsin inhibitor in the concentrated benzamidine eluate. The antisera to alpha two-macroglobulin gave an immunoprecipitate which was readily stained for proteolytic activity. On immunoelectrophoresis, the alpha two-macroglobulin precipitin band corresponded to the band absent in plasma of patients with cystic fibrosis. In contrast, the alpha two-macroglobulin levels were similar in plasma of control subjects and patients with cystic fibrosis. Using the antiserum to the protein fractith proteolytic activity could be demonstrated in control plasma. One specific enzyme-active "rocket" was absent in plasma of patients with cystic fibrosis. In a double blind study of 15 control samples and 15 samples from patients with cystic fibrosis, a specific "rocket" was shown to be present in 13 control samples and absent in 14 cystic fibrosis samples. alpha two-Macroglobulin was determined by both an immunologic procedure and by its trypsin binding (trypsin protein esterase concentration). The ratio of the immunologic assay to the biologic activity assay was 90 for the normal plasma samples and only 65 for cystic fibrosis samples.
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PMID:Absence of an alpha two-macroglobulin-protease complex in cystic fibrosis. 6 Jul 35

Because immediate hypersensitivity reactions can occur in individuals exposed to powdered pancreatic extracts, 36 patients with cystic fibrosis and 51 patents of such patients wwer studied for evidence of sensitization. Sensitivity to the extracts as evidence by history and skin testing was infrequent in the children with cystic fibrosis. However, skin testing for immediate hypersensitivity with either crude pancreatic extracts or inactivated trypsin correlated well in their patents with a history of clinical symptoms. IgE mediation of these reactions in sensitized individuals was demonstrated by antigen-induced histamine release from leukocytes, passive transfer studies, and immediate response to inhalation challenge.
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PMID:Hypersensitivity to pancreatic extracts in parents of patients with cystic fibrosis. 6 83

The complex of trypsin with purified alpha2-macroglobulin from normals and patients with cystic fibrosis was studied. The formed complex failed to reveal any proteolytic activity toward a high molecular weight substrate whereas the esterolytic activity towards a low molecular weight substrate was retained. This esterolytic activity was resistant to inhibition by a high molecular weight inhibitor. During iincubation at 38 degrees C the complex with normal alpha2-macroglobulin was slowly inhibited by the high molecular weight inhibitor and regained activity with the high molecular weight substrate. This phenomenon was not obtained when the alpha2-macroglobulin from cystic fibrosis was examined. These data suggest that the gradual conversion of normal alpha2-macroglobulin-trypsin complex into an alpha2-macroglobulin fragment-trypsin complex is deficient in patients with cystic fibrosis.
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PMID:Abnormal breakdown of alpha2-macroglobulin-trypsin complex in cystic fibrosis. 6 10

In a new method of testing stool samples from newborn babies for cystic fibrosis (C.F.), a colourless substrate, benzoyl-arginine-p-nitroanilide (B.A.P.N.A.), releases yellow p-nitroaniline when hydrolysed by trypsin. Samples from infants with C.F., who lack trypsin, give negligible colour. 2 infants with C.F. were detected among 2500 consecutive newborn babies tested. The incidence of false-positive results was 1.2% after the first specimen and 0.05% after the second specimen. A further refinement has reduced the positive rate to 0.1% after the first specimen (2000 samples). Tests on samples from 5 other older patients with untreated C.F. have yielded no evidence for false-negative results.
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PMID:Cystic-fibrosis screening in the newborn. 7 7

Serum-immunoreactive-trypsin (I.R.T.) was measured in children with cystic fibrosis (C.F.) and a variety of controls. In the first few months of life all C.F. children had a raised serum-I.R.T. A dried blood-spot assay for I.R.T. was established and has potential as a screening test for C.F. in the newborn.
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PMID:Dried-blood spot screening for cystic fibrosis in the newborn. 8 57

On exposure to pancreatic extract (PE), four parents of cystic fibrosis (CF) children developed immediate hypersensitivity-like symptoms: i.e. rhinoconjunctivitis, asthma, and/or anaphylaxis. IgE to PE was demonstrated in the subjects by skin testing, leucocyte histamine release and radioallergosorbent test (RAST). No serum precipitating antibodies were found. Bronchial challenge caused an immediate asthmatic response. No delayed asthmatic response or hypersensitivity pneumonitis-like reaction occurred. The responsible antigen for PE induced asthma is unknown; trypsin failed to inhibit PE-RAST and is therefore not responsible in these subjects. Caution should be exercised in using PE for skin testing and bronchial challenge in subjects with suspected hypersensitivity to PE. Certain measures were found useful in preventing the occurrence of symptoms in the four subjects.
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PMID:IgE mediated hypersensitivity to pancreatic extract (PE) in parents of cystic fibrosis (CF) children. 8 85

Samples of plasma or serum from 53 cystic fibrosis (CF) patients, 90 relatives of CF patients , and 159 controls have been incubated with porcine or bovine 125I-trypsin, electrophoresed on polyacrylamide gel, and autoradiographed. In these individuals, the main binding protein for 125I-trypsin has been shown to be alpha 2-macroglobulin (alpha 2M). Using this method of analysis, no difference in electrophoretic migration of 125I-trypsin-alpha 2M complexes has been observed between CF and control individuals. However, trypsin binding to IgG has been observed in 80% of CF patients, 30% of their mothers, 3% of controls, and in two patients affected with pancreatitis. These trypsin binding immunoglobulins are called TbIg, and specifically, Tb1gG when referring to the G class. Experimental evidence indicates that binding of trypsin to IgG occurs through the Fab portion of the molecule. Tb1gG must be antibodies most probably induced by the exogenous trypsin ingested daily by most CF patients (and by patients affected with chronic pancreatitis). Antibodies against porcine pancreatic elastase have been observed using the same analysis, but not as frequently as Tb1g.
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PMID:Binding of 125I-labeled proteinases to plasma proteins in cystic fibrosis. 9 5

An immunoreactive-trypsin assay uses small dried-blood spots (diameter 1.25 mm) and is therefore suitable for incorporation in established neonatal screening schemes. Blood specimens from neonates with cystic fibrosis had trypsin levels greater than those in control subjects, thus confirming earlier findings. Trypsin levels were below normal in several older patients with cystic fibrosis.
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PMID:Sensitive trypsin assay for dried-blood specimens as a screening procedure for early detection of cystic fibrosis. 9 25

Immunochemical and functional properties of control and Cystic Fibrosis (CF) alpha 2-Macroglobulin (alpha 2M) are compared. Crossed immunoelectrophoresis and Ouchterlony double diffusion revealed no qualitative differences between the two alpha 2-M preparations. Trypsin-esterase activity assayed with BAPNA as a substrate, in the presence of an excess STI, gave similar ratios between total and active alpha 2M. These alpha 2M-trypsin complexes were equally stable under various experimental conditions and maintained a constant STI non-inhibited esterase activity. Normal and CF-alpha 2M-trypsin complexes were taken up by normal human fibroblasts to a similar extent during a four hour period. The only significant difference was observed when the uptake of alpha 2M from untreated sera was examined. The uptake of alpha 2M from CF sera was always lower than from pooled control sera despite large variation. Mixing of control and CF serum did not affect the normal uptake and other serum components were taken up to the normal extent. Intracellular degradation of CF alpha 2M had a half life of 2.0 to 2.8 hours, which compares well to the normal half life of 2.2 hours. More work needs to be done on the nature of the interaction between alpha 2M and proteases before a reasonable explanation for the molecular nature of the abnormal behavior can be sought.
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PMID:Anomalous alpha 2-macroglobulin-protease complexes in cystic fibrosis: decreased uptake of the complexes by fibroblasts in culture. 9 65

The esterolytic activity of mixed and parotid saliva in cystic fibrosis (CF) patients and normal subjects was determined using BAEE (alpha-Benzoyl-1-arginine-ethylester) as the substrate. Using soybean-trypsin-inhibitor the trypsin-like activity (TLA) was measured and plotted as a function of parotid flow rate. In addition calcium, protein and pH were determined. Trypsin-like activity in mixed and parotid saliva showed large individual variations in CF and normal children. In parotid saliva we could not find any significant difference, whereas a reduction of TLA in mixed saliva of CF patients was observed. The fact that our normal values fell within the range of heterozygotes reported by Rao et al. (19), makes their hypothesis of a close relationship between reduced TLA and the genetic defect very doubtful. Protein, calcium and pH increased with augmented salivation and no difference between CF patients and normal age matched children could be found except for the pH at a flow rate above 0.75 ml/min per m2 body surface where significantly lower pH values resulted. The relevance of reduced TLA to the pathogenesis of cystic fibrosis is discussed.
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PMID:Excretion of trypsin-like activity, electrolytes and protein in mixed and parotid saliva of patients with cystic fibrosis of the pancreas. 23 50


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