Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0272170 (SDS)
 50,377 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ten lectins have been used to detect glycoproteins, after SDS polyacrylamide gel electrophoresis and gel isoelectric focusing, in fibroblasts, red cell membranes, urine, and plasma of patients and obligate heterozygotes with cystic fibrosis. No disease specific changes were detected but considerable individual variation was observed, some of which was attributed to known genetic polymorphisms unrelated to cystic fibrosis.
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PMID:Glycoproteins in cystic fibrosis: a lectin binding study. 651 32

Previous studies have shown that human small-intestinal mucin consists of high-Mr glycoproteins and a smaller S-S-bonded protein of 118 kDa. The major antigenic determinants of the mucin were associated with the large glycoproteins, but depended for stability on intact disulphide bonds, and were destroyed by digestion with Pronase. In the present study we isolated and analysed the component parts of mucin from patients with cystic fibrosis with special attention being paid to the peptide constituents. After reduction with 0.2 M-beta-mercaptoethanol [5 min, 100 degrees C in 1% SDS (sodium dodecyl sulphate)], the large glycoproteins and smaller peptide with an apparent molecular size of 118 kDa were separated by equilibrium density-gradient centrifugation in CsCl, Sepharose 4B chromatography or preparative SDS/polyacrylamide-gel electrophoresis. The large glycoproteins contained about 70% of the protein of the native mucin. Digestion with Pronase resulted in a further loss of 'naked' protein (10% of the native mucin protein) from the C-terminal end of the glycoprotein peptide core, and left behind highly glycosylated proteins comprised mainly (70 mol%) of threonine, serine and proline. The 118 kDa component, which contained about 30% of the native mucin protein, consisted mainly of aspartic acid, serine, glutamic acid and glycine (40 mol%), plus threonine, proline, alanine, valine and leucine (35 mol%). Together with the 'naked' protein segment, the 118 kDa component contained most of the cysteine residues of the native mucin. Surprisingly, the peptide also contained carbohydrate (less than or equal to 5% of the native mucin carbohydrate but 50% by weight of the 118 kDa component), which included 9 mol% mannose, suggesting the presence of N-linked oligosaccharides. The peptide exhibited strong non-covalent interactions with the high-Mr glycoproteins and a tendency to self-aggregate in the absence of dissociating agents. Our findings therefore suggest that native mucin consists of large glycoproteins capable of forming disulphide bridges from their C-terminal 'naked' (antigenic) regions to a smaller glycopeptide having an Mr of 118 000.
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PMID:Biochemical characterization of the component parts of intestinal mucin from patients with cystic fibrosis. 651 57

Changes in the protease activity in amniotic fluid has been proposed as a valid method for the prenatal detection of cystic fibrosis (CF). We have studied by quantitative and qualitative procedures, sixty four amniotic fluids: two of them from CF-affected fetuses. Interpretation of the benzoyl arginine ethyl ester (BAEE)-staining patterns after isoelectric focusing was often difficult, and repeated experiments gave variable results. In order to improve gel discrimination, we performed amniotic fluid electrofocusing in the presence of detergents: 0.1 per cent Triton X-100, 0.1 per cent DOC, or 0.1 per cent SDS. In these conditions, the pattern revealed by BAEE was modified, but no differences were observed between CF and normal amniotic fluids.
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PMID:Amniotic fluid protease activity and the prenatal detection of cystic fibrosis. 662 98

4-methylumbelliferylguanidinobenzoate (MUGB) reactivity in plasma from patients with cystic fibrosis and in amniotic fluid from pregnancies leading to children with cystic fibrosis, has been reported to be significantly decreased. We have so far been unable to confirm these findings and have therefore reexamined this reactivity using diisopropylfluorophosphate (DEF), another active site titrant of serine proteases. We have shown that MUGB and DFP are reacting with the same molecules in plasma and amniotic fluid. Using crossed immunoelectrophoresis and SDS-polyacrylamide gel electrophoresis of 3H-DFP labelled plasma and amniotic fluid we have obtained strong evidence that the main contribution of MUGB and DFP reactive protein in plasma and amniotic fluid is identical to serum albumin. The use of MUGB reactivity in amniotic fluid in pregnancies at risk for cystic fibrosis must therefore be reconsidered.
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PMID:A serine protease activity of human serum albumin towards 4-methylumbelliferyl-guanidinobenzoate (MUGB) and diisopropyl fluorophosphate (DEP): implications for the use of MUGB reactivity in amniotic fluid in prenatal diagnosis of cystic fibrosis. 675 41

Heparin binding to serum proteins and their subsequent precipitation is reportedly increased in cystic fibrosis (CF). We have confirmed this finding for CF patients over the age of 12 [11.34 +/- 2.42 mg/ml precipitated protein for normals (n = 19) versus 17.46 +/- 4.60 mg/ml for CF patients (n = 37), P less than 0.001; 0.629 +/- 0.098 mg/ml precipitated heparin for normals versus 0.789 +/- 0.206 for CF patients, P less than 0.01]. We have also shown that patients with a variety of pulmonary diseases unrelated to CF do not show this effect. When the amounts of protein and heparin precipitated are compared with the amount of IgG found in the whole serum sample, the correlation coefficients (protein r = 0.77; heparin, 0.74; n = 81) are significant at a level of P less than 0.001. In addition, the report that young CF patients exhibit hypogammaglobulinemia prompted us to examine serum samples from CF patients and age-matched controls under the age of 12. No differences were found. To investigate the molecular basis for this effect, sera from patients with CF and from age-matched controls were precipitated with 50 mg% heparin at pH 5.57. Pellets resolubilized in 8 M urea were fractionated on DEAE-Sephadex and analyzed by double-immunodiffusion, SDS-PAGE, immunoelectrophoresis, and radial immunodiffusion. IgG constituted 55-56% of the eluted protein. When serum from all donors was fractionated by Staph A-Sepharose into IgG and non-IgG fractions, 85-89% of heparin precipitable protein was in the IgG fraction.
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PMID:Identification of a major heparin-precipitable protein in human serum and its relationship to cystic fibrosis. 706 73

The absorption of vitamin B12, labelled with radioactive 58Co, was measured in 19 patients with cystic fibrosis and one child with the Shwachman-Diamond syndrome using the whole body counting technique. We found vitamin B12 absorption reduced to 7.97 on average, compared to 59.2% for the control group. The low vitamin B12 absorption correlated well with the reduced fat retention coefficients. After adding 0.212 pancreatin to the radioactive vitamin B12 test dose, the absorption quotas improved in all cases, the average being 61%. A meal poor in vitamin B12 tended to increase the absorption of the radioactive test dose to 23% on average. As yet there is no satisfactory explanation for the effect of the diet on the absorption of vitamin B12 in exocrine pancreatic insufficiency. This could be the reason why the malabsorption of vitamin B12 in patients with EPI can go unnoticed for many years and could possibly explain why vitamin B12 malabsorption in exocrine pancreatic insufficiency does not cause symptoms and signs of vitamin B12 deficiency for many years.
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PMID:Vitamin B12 absorption and exocrine pancreatic insufficiency in childhood. 721 91

Plasma immunoreactive cationic trypsin(ogen) levels were determined in 32 control subjects and 43 patients with varying degrees of pancreatic insufficiency including 35 with cystic fibrosis (CF) and eight with Shwachman's syndrome. In six CF infants less than 2 years of age, plasma trypsin(ogen) levels were significantly elevated (97.3 +/- 62.2 ng/ml) above the normal range for nine controls (7.0 +/- 5.9 ng/ml; P less than 0.025). Four of these infants had steatorrhea, three of whom had undetectable duodenal trypsin activity after stimulation with secretin-cholecystokinin. In two CF infants, molecular size fractionation by gel filtration of plasma followed by radioimmunoassay of the column fractions demonstrated that trypsinogen was the only immunoreactive species in the circulation. In contrast, in older CF patients with steatorrhea (mean age, 15.3 +/- 4.6 years), plasma cationic trypsin(ogen) levels were undetectable or low (1.1 +/- 1.7 ng/ml). This finding clearly distinguished them from older CF patients without steatorrhea (mean age, 14.3 +/- 3.9 years) in whom cationic trypsin(ogen) levels were significantly higher (23.3 +/- 17.6 ng/ml; P less than 0.01). The mean trypsin(ogen) concentration in the older CF patients without steatorrhea did not differ from the mean value for 23 normal subjects of similar age. Plasma cationic trypsin(ogen) levels in two Schwachman's patients with steatorrhea (0.19 and 0.86 ng/ml) were significantly lower than the values found in six Shwachman's patients without steatorrhea (5.9 +/- 2.3 ng/ml; P less than 0.025). Furthermore, in nine older CF patients and eight Schwachman's patients, circulating trypsin(ogen) levels were highly correlated with duodenal trypsin output after secretin-cholecystokinin stimulation (r = 0.946, P less than 0.01; r = 0.899, P less than 0.01, respectively). These results suggest that in CF infants high levels of circulating trypsin(ogen) persist even in those with Shwachman's syndrome, however, circulating trypsin(ogen) accurately reflects residual pancreatic function.
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PMID:Plasma immunoreactive pancreatic cationic trypsinogen in cystic fibrosis: a sensitive indicator of exocrine pancreatic dysfunction. 730 57

Representative isolates of Pseudomonas cepacia from 15 cystic fibrosis (CF) patients attending the Respiratory Unit of Alder Hey Childrens' Hospital were investigated by SDS-PAGE of whole-cell polypeptides and by pyrolysis mass spectroscopy (PMS). SDS-PAGE was less discriminatory than PMS. Eleven isolates were indistinguishable by PMS and considered to represent re-isolates of an endemic strain; four isolates were distinct from this group, and from one another. P. cepacia was first isolated on the unit in July 1989 from a patient who had attended a UK selection meeting for a Canadian CF camp. A ward and outpatient segregation policy was introduced, but colonisation of further patients occurred. In August 1991, the Adult CF Association recommended that all social activities involving colonised patients should cease. This, and an increased awareness amongst older CF patients of the risks of person-to-person transmission, was associated with a marked decline in new cases. Social activity and hospital admissions were compared for colonised patients during the year before colonisation with P. cepacia, and matched patients who did not acquire the endemic strain. This showed a significantly higher attendance at CF social events for colonised patients, but no significant association between colonisation and hospital admission. These results are strong indirect evidence that transmission of P. cepacia occurs through social contact outside the hospital environment.
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PMID:Application of pyrolysis mass spectroscopy and SDS-PAGE in the study of the epidemiology of Pseudomonas cepacia in cystic fibrosis. 751 69

Burkholderia cepacia (Pseudomonas cepacia) is now recognised as an important pathogen in cystic fibrosis patients, and several reports have suggested that sputum-culture-proven colonisation occurs despite the presence of specific antibody. In an attempt to establish the use of antibody studies as diagnostic and prognostic indicators of B. cepacia infection, we have examined the IgG response to B. cepacia outer membrane proteins and lipopolysaccharide in patients also colonised with P. aeruginosa. The B. cepacia strains were grown in a modified iron-depleted chemically defined medium and outer membrane components examined by SDS-PAGE and immunoblotting. IgG antibodies were detected against B. cepacia outer membrane antigens, which were not diminished by extensive preadsorption with P. aeruginosa. The response to B. cepacia O-antigen could be readily removed by adsorption of serum either with B. cepacia whole cells or purified LPS, whereas we were unable to adsorb anti-outer membrane protein antibodies using B. cepacia whole cells. The inability to adsorb anti-outer membrane protein antibodies using B. cepacia whole cells maybe due to non-exposed surface epitopes. Several B. cepacia sputum-culture negative patients colonised with P. aeruginosa had antibodies directed against B. cepacia outer membrane protein. this study suggests that there is a specific anti-B. cepacia LPS IgG response, which is not due to antibodies cross-reactive with P. aeruginosa. Our studies indicate that much of the B. cepacia anti-outer membrane protein response is specific and not attributable to reactivity against co-migrating LPS.
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PMID:Serum IgG response to Burkholderia cepacia outer membrane antigens in cystic fibrosis: assessment of cross-reactivity with Pseudomonas aeruginosa. 753 70

The expression patterns of a variety of cytoskeletal antigens were studied in normal human tissues (placenta, umbilical cord, myometrium, colon, mammary gland, testis, skeletal muscle, myocardium) as well as in abnormal human tissues (palmar fibromatosis, fibrocystic disease of the mammary gland, mammary carcinoma). The immunohistochemical binding patterns of the monoclonal antibody GB 42 were compared to those of commercial antibodies directed against vimentin, desmin, smooth muscle myosin, pan actin, alpha-smooth muscle actin and gamma-smooth muscle actin. Methods applied comprised immunohistochemistry on cryostat sections and paraffin sections. Immunogold immunocytochemistry was performed on Lowicryl sections. The patterns of GB 42-binding were confirmed biochemically by SDS-PAGE and Western-blotting, and quantitative amino acid analysis. Our data suggest that the monoclonal antibody GB 42 recognizes an actin isoform which is identical to, or closely related to, gamma-smooth muscle actin. Unlike the commercially available antibody against gamma-smooth muscle actin, GB 42 does not cross-react with alpha-skeletal or alpha-cardiac actins. The GB 42-antigen is expressed in smooth muscle cells, myoepithelial cells and in later stages of differentiation of myofibroblasts, in all the tissues investigated. Throughout the development of smooth muscle cells and myofibroblasts, the appearance of the GB 42-antigen occurs after the expression of vimentin, desmin and alpha-smooth muscle actin, but prior to the expression of smooth muscle myosin. GB 42 is a reliable marker for higher stages of differentiation of smooth muscle cells and myofibroblasts.
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PMID:The monoclonal antibody GB 42--a useful marker for the differentiation of myofibroblasts. 764 18


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