Cystic fibrosis and CFTR

Greger, R, Schreiber, R, Mall, M, Wissner, A, Hopf, A, Briel, M, Bleich, M, Warth, R and Kunzelmann, K (2001) Cystic fibrosis and CFTR. Pflugers Archiv-european Journal of Physiology, 443 1: S3-S7.

Author Greger, R
Schreiber, R
Mall, M
Wissner, A
Hopf, A
Briel, M
Bleich, M
Warth, R
Kunzelmann, K
Title Cystic fibrosis and CFTR
Journal name Pflugers Archiv-european Journal of Physiology   Check publisher's open access policy
ISSN 0031-6768
Publication date 2001
Sub-type Article (original research)
Volume 443
Issue 1
Start page S3
End page S7
Total pages 5
Editor K Thurau
Place of publication Germany
Publisher Springer - Verlag
Collection year 2001
Language eng
Subject C1
270104 Membrane Biology
730110 Respiratory system and diseases (incl. asthma)
Abstract Cystic fibrosis (CF) is a complex disease affecting epithelial ion transport. There are not many diseases like CF that have triggered such intense research activities. The complexity of the disease is due to mutations in the CFTR protein, now known to be a Cl- channel and a regulator of other transport proteins. The various interactions and the large number of disease-causing CFTR mutations is the reason for a variable genotype-phenotype correlation and sometimes unpredictable clinical manifestation. Nevertheless, the research of the past 10 years has resulted in a tremendous increase in knowledge, not only in regard to CFTR but also in regard to molecular interactions and completely new means of ion channel and gene therapy.
Keyword Physiology
Cystic Fibrosis
Cftr
Cystic Fibrosis Transmembrane Conductance Regulator
Enac
Epithelial Na+ Channel
Epithelium
Airways
Intestine
Transmembrane Conductance Regulator
Inhibitable Na+ Conductance
Activated Cl Conductance
Airway Epithelial-cells
Hamster Ovary Cells
Xenopus Oocytes
Anion Selectivity
Expressing Cftr
Colonic Crypts
Channel
Q-Index Code C1

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Biomedical Sciences Publications
 
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Created: Tue, 14 Aug 2007, 15:11:17 EST