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cystic fibrosis/proline
Veza se sprema u međuspremnik
Stranica 1 iz 61 rezultatima
Contribution of proline residues in the membrane-spanning domains of cystic fibrosis transmembrane conductance regulator to chloride channel function.
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Proline residues located in membrane-spanning domains of transport proteins are thought to play an important structural role. In the cystic fibrosis transmembrane conductance regulator (CFTR), the predicted transmembrane segments contain four prolines: Pro99, Pro205, Pro324, and Pro1021. These
Proline-Glycine-Proline (PGP) and High Mobility Group Box Protein-1 (HMGB1): Potential Mediators of Cystic Fibrosis Airway Inflammation.
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Cystic fibrosis (CF) is chronic lung disease characterized by an unrelenting neutrophil-predominant airway inflammatory response. This inflammation leads to extracellular matrix (ECM) remodeling and eventually to the development of bronchiectasis. While many components of the immune response in CF
Proline-glycine-proline as a potential biomarker in chronic obstructive pulmonary disease and cystic fibrosis.
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The present review discusses the role of tri-peptide Proline -Glycine -Proline (PGP) as a potential player, biomarker and therapeutic target in this process.
A serine to proline substitution (S1255P) in the second nucleotide binding fold of the cystic fibrosis gene.
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Studies in cystic fibrosis; determination of plasma proline following protein feeding as a diagnostic test for pancreatic insufficiency.
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Benzyloxycarbonyl-proline-prolinal (ZPP): Dual complementary roles for neutrophil inhibition.
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Neutrophil influx and activation contributes to organ damage in several major lung diseases. This inflammatory influx is initiated and propagated by both classical chemokines such as interleukin-8 and by downstream mediators such as the collagen fragment cum neutrophil chemokine Pro-Gly-Pro (PGP),
Potential role of high-mobility group box 1 in cystic fibrosis airway disease.
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BACKGROUND
High-mobility group box 1 (HMGB1) is a potent inflammatory mediator elevated in sepsis and rheumatoid arthritis, although its role in cystic fibrosis (CF) lung disease is unknown.
OBJECTIVE
To determine whether HMGB1 contributes to CF lung inflammation, including neutrophil chemotaxis and
Mucinophilic and chemotactic properties of Pseudomonas aeruginosa in relation to pulmonary colonization in cystic fibrosis.
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Representative isolates of nonmucoid Pseudomonas aeruginosa were studied to investigate the hypothesis that mucinophilic and chemotactic properties in this species act as potential factors in the initial stages of pulmonary colonization in patients with cystic fibrosis (CF). Transmission electron
Biochemical characterization of the component parts of intestinal mucin from patients with cystic fibrosis.
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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
Destabilization of the transmembrane domain induces misfolding in a phenotypic mutant of cystic fibrosis transmembrane conductance regulator.
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Two phenotypic missense mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) channel pore (L346P and R347P in transmembrane (TM) segment 6) involve gain of a proline residue, but only L346P represents a significant loss of segment hydropathy. We show here that, for synthetic
Molecular analysis of the cystic fibrosis gene reveals a high frequency of the intron 8 splice variant 5T in Egyptian males with congenital bilateral absence of the vas deferens.
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It has previously been shown that defects in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are largely responsible for the condition of congenital bilateral absence of the vas deferens (CBAVD), without associated renal abnormalities, in Caucasian populations. To assess the
Colloid and crystal formation in parotid saliva of cystic fibrosis patients and non-cystic fibrosis subjects. II. Electron microscopy and electrophoresis.
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Centrifuged pellets of turbid parotid saliva from cystic fibrosis (CF) patients and non-CF subjects, obtained from saliva kept at 2 degrees for 10 min, had the electron microscope appearance of amorphous, round particles, and were thought to be colloidal aggregates of organic material. Drops of
Proline-rich proteins are present in serous cells of submucosal glands in the respiratory tract.
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Using antibodies to basic and acidic proline-rich proteins (PRP) of salivary origin, we detected PRP immunoreactivity in serous cells of human nasal, laryngeal, and tracheobronchial glands by an immunoperoxidase technique. Immunoreactive PRP, detected by immunoblotting from SDS gels, were also found
Conserved allosteric hot spots in the transmembrane domains of cystic fibrosis transmembrane conductance regulator (CFTR) channels and multidrug resistance protein (MRP) pumps.
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ATP-binding cassette (ABC) transporters are an ancient family of transmembrane proteins that utilize ATPase activity to move substrates across cell membranes. The ABCC subfamily of the ABC transporters includes active drug exporters (the multidrug resistance proteins (MRPs)) and a unique ATP-gated
Intermolecular interaction between R domains of cystic fibrosis transmembrane conductance regulator.
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The function of the R domain of cystic fibrosis transmembrane conductance regulator (CFTR) has not yet been fully established. The cis-trans proline isomerase cyclophilin A stimulates channel activity, and stimulation depends on the presence of highly conserved prolines at positions 740, 750, and
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