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Keywords: cystic fibrosis
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Articles
The multiple ubiquitination mechanisms in CFTR peripheral quality control
Available to Purchase
Journal:
Biochemical Society Transactions
Biochem Soc Trans (2023) 51 (3): 1297–1306.
Published: 04 May 2023
...Shogo Taniguchi; Ryosuke Fukuda; Tsukasa Okiyoneda The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-regulated anion channel, which is expressed on the apical plasma membrane (PM) of epithelial cells. Mutations in the CFTR gene cause cystic fibrosis (CF), one of the most...
Articles
A ‘tad’ of hope in the fight against airway disease
Available to Purchase
Journal:
Biochemical Society Transactions
Biochem Soc Trans (2020) 48 (5): 2347–2357.
Published: 20 October 2020
... ) 12 8 2020 21 9 2020 22 9 2020 © 2020 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society 2020 asthma chronic obstructive pulmonary disease cilia cystic fibrosis mucus Xenopus As ciliated cells differentiate, cilia are generated...
Articles
Non-coding RNA in cystic fibrosis
Available to Purchase
Journal:
Biochemical Society Transactions
Biochem Soc Trans (2018) 46 (3): 619–630.
Published: 09 May 2018
... based on their size or function. Two of the more well-known and widely studied ncRNA species are microRNAs (miRNAs) and lncRNAs. These are regulatory RNAs and their altered expression has been implicated in the pathogenesis of a variety of human diseases. Failure to express a functional cystic fibrosis...
Articles
Exploiting species differences to understand the CFTR Cl − channel
Available to Purchase
Journal:
Biochemical Society Transactions
Biochem Soc Trans (2015) 43 (5): 975–982.
Published: 09 October 2015
...Samuel J. Bose; Toby S. Scott-Ward; Zhiwei Cai; David N. Sheppard The anion channel cystic fibrosis transmembrane conductance regulator (CFTR) is a unique ATP-binding cassette (ABC) transporter. CFTR plays a pivotal role in transepithelial ion transport as its dysfunction in the genetic disease...
Includes: Supplementary data
Articles
Distribution of human PLUNC/BPI fold-containing (BPIF) proteins
Available to Purchase
Journal:
Biochemical Society Transactions
Biochem Soc Trans (2011) 39 (4): 1023–1027.
Published: 20 July 2011
... Society 2011 cystic fibrosis eutherian lineage mucous cell non-small cell lung carcinoma palate lung and nasal epithelium clone (PLUNC) BPI bactericidal/permeability-increasing protein BPIF BPI fold CF cystic fibrosis NPC nasopharyngeal carcinoma NSCLC non-small...
Articles
The bactericidal/permeability-increasing protein (BPI) in the innate defence of the lower airways
Available to Purchase
Journal:
Biochemical Society Transactions
Biochem Soc Trans (2011) 39 (4): 1045–1050.
Published: 20 July 2011
... defence molecules such as BPI, are of exceptional relevance. In the present review, we highlight possible functions of BPI during acute pneumonia and CF (cystic fibrosis)-associated chronic infections in the lung. 1 To whom correspondence should be addressed (email [email protected]...
Articles
Genistein: a natural isoflavone with a potential for treatment of genetic diseases
Available to PurchaseGrzegorz Węgrzyn, Joanna Jakóbkiewicz-Banecka, Magdalena Gabig-Cimińska, Ewa Piotrowska, Magdalena Narajczyk, Anna Kloska, Marcelina Malinowska, Dariusz Dziedzic, Izabela Gołębiewska, Marta Moskot, Alicja Węgrzyn
Journal:
Biochemical Society Transactions
Biochem Soc Trans (2010) 38 (2): 695–701.
Published: 22 March 2010
... review, we discuss a plausible use of genistein in treatment of two genetic disorders: CF (cystic fibrosis) and MPS (mucopolysaccharidosis). Although various biological actions of genistein are employed in these two cases, in vitro studies, tests on animal models and pilot clinical trials suggest...
Articles
Role of CFTR, Pseudomonas aeruginosa and Toll-like receptors in cystic fibrosis lung inflammation
Available to Purchase
Journal:
Biochemical Society Transactions
Biochem Soc Trans (2009) 37 (4): 863–867.
Published: 22 July 2009
...Paul J. Buchanan; Robert K. Ernst; J. Stuart Elborn; Bettina Schock CF (cystic fibrosis) is a severe autosomal recessive disease most common in Northwest European populations. Underlying mutations in the CFTR (CF transmembrane conductance regulator) gene cause deregulation of ion transport...
Articles
Clinical potential of mannose-binding lectin-replacement therapy
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Journal:
Biochemical Society Transactions
Biochem Soc Trans (2003) 31 (4): 770–773.
Published: 01 August 2003
... subjects with MBL deficiency remain healthy. MBL deficiency is also associated with non-infectious diseases including systemic lupus erythematosus, rheumatoid arthritis, cystic fibrosis and common variable immunodeficiency. MBL deficiency may affect susceptibility to (e.g. meningococcal disease), or alter...