...Michelle L. McClure; Hui Wen; James Fortenberry; Jeong S. Hong; Eric J. Sorscher Defects in CFTR (cystic fibrosis transmembrane conductance regulator) maturation are central to the pathogenesis of CF (cystic fibrosis). Palmitoylation serves as a key regulator of maturational processing in other...

...Kendra Tosoni; Michelle Stobbart; Diane M. Cassidy; Andrea Venerando; Mario A. Pagano; Simão Luz; Margarida D. Amaral; Karl Kunzelmann; Lorenzo A. Pinna; Carlos M. Farinha; Anil Mehta Most CF (cystic fibrosis) results from deletion of a phenylalanine (F 508 ) in the CFTR {CF transmembrane...

...Mario A. Pagano; Oriano Marin; Giorgio Cozza; Stefania Sarno; Flavio Meggio; Kate J. Treharne; Anil Mehta; Lorenzo A. Pinna Cystic fibrosis mostly follows a single Phe 508 deletion in CFTR (cystic fibrosis transmembrane regulator) (CFTRΔF508), thereby causing premature fragmentation of the nascent...

... of innate immunity. In cystic fibrosis, a hereditary disease with frequent lung infection with Pseudomonas aeruginosa , the non-esterified fatty acid DA (docosahexaenoic acid), but not OA (oleic acid), is decreased, and DA supplementation has been shown to improve the clinical condition in these patients...

...Luba Aleksandrov; Andrei Aleksandrov; John R. Riordan ATP binding to the first and second NBDs (nucleotide-binding domains) of CFTR (cystic fibrosis transmembrane conductance regulator) are bivalent-cation-independent and -dependent steps respectively [Aleksandrov, Aleksandrov, Chang and Riordan...

...Emanuela Caci; Antonella Caputo; Alexandre Hinzpeter; Nicole Arous; Pascale Fanen; Nitin Sonawane; A. S. Verkman; Roberto Ravazzolo; Olga Zegarra-Moran; Luis J. V. Galietta CFTR (cystic fibrosis transmembrane conductance regulator) is an epithelial Cl − channel inhibited with high affinity...

...Tip W. Loo; M. Claire Bartlett; David M. Clarke Cystic fibrosis (CF) is most commonly caused by deletion of a residue (ΔF508) in the CFTR (cystic fibrosis transmembrane conductance regulator) protein. The misfolded mutant protein is retained in the ER (endoplasmic reticulum) and is not trafficked...

...Sophie Groux-Degroote; Marie-Ange Krzewinski-Recchi; Aurélie Cazet; Audrey Vincent; Sylvain Lehoux; Jean-Jacques Lafitte; Isabelle van Seuningen; Philippe Delannoy Bronchial mucins from patients suffering from CF (cystic fibrosis) exhibit glycosylation alterations, especially increased amounts...

... Journal show that, for mutant forms of two ABC (ATP-Binding-Cassette) transporters, P-glycoprotein and CFTR (cystic fibrosis transmembrane conductance regulator), modest correction of trafficking by single agents can be additive when multiple compounds are used in combination. These findings raise...

... immunity in the lung, and have been reported to be altered in lung disease. Changes in lung mucosal glycosylation have been reported in CF (cystic fibrosis), which may be responsible for differential bacterial binding to glycosylated components in the lung mucosa and hence increased pre-disposition...

...) concentrations of the endogenous S-nitrosothiol, GSNO ( S -nitrosoglutathione), on the activities of nuclear regulatory proteins Sp3 and Sp1 (specificity proteins 3 and 1). Low concentrations of GSNO increased Sp3 binding, as well as Sp3-dependent transcription of the cystic fibrosis transmembrane conductance...

... cell expressed developmentally down-regulated 4) is of central importance. Other mechanisms known to interact with ENaC and affect sodium transport include channel-activating protease 1 (CAP-1), a membrane-anchored protein, and the cystic fibrosis transmembrane regulator. The implications of research...

..., London ©2002 2002 bacterial toxin cytotoxicity cystic fibrosis GTP-binding protein Pseudomonas aeruginosa Abbreviations used: ARF, ADP-ribosylation factor; BD, binding domain; ExoS, exoenzyme S; ExoT, exoenzyme T; FCS, fetal calf serum; GAP, GTPase-activating protein; GDS, GDP/GTP...

... and spontaneous sputa from asthmatic airways. Additionally we have used this procedure to analyse these glycoproteins in mucin preparations purified from cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) mucus. Our findings indicate that MUC5AC and MUC5B are the major oligomeric mucins...

... of cystic fibrosis and other secretory disorders of the liver and other epithelial tissues. 1 To whom correspondence should be sent, at the following address: Digestive Diseases Section, Room 1080 LMP, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06520-8019, U.S.A. (e...

...-ribosylation cystic fibrosis ExoS Pseudomonas aeruginosa. Biochem. J. (2000) 349, 697 701 (Printed in Great Britain) 697 RESEARCH COMMUNICATION 14-3-3 proteins are required for the inhibition of Ras by exoenzyme S Maria Lena HENRIKSSON, Ulrika TROLLE! R and Bengt HALLBERG1 Cellular and Molecular Biology...

...Julia R. DAVIES; Naila SVITACHEVA; Louise LANNEFORS; Ragnhild KORNFÄLT; Ingemar CARLSTEDT To investigate the genetic identities of the mucins secreted in cystic fibrosis (CF) airways, sputum was collected from five individuals. Samples were separated into gel and sol phases by high-speed...

...Changsen WANG; Ariel F. CASTRO; Denise M. WILKES; Guillermo A. ALTENBERG Many membrane proteins that belong to the ATP-binding cassette (ABC) superfamily are clinically important, including the cystic fibrosis transmembrane conductance regulator, the sulphonylurea receptor and P-glycoprotein...