Lung infections associated with cystic fibrosis

JB Lyczak, CL Cannon, GB Pier - Clinical microbiology reviews, 2002 - Am Soc Microbiol
While originally characterized as a collection of related syndromes, cystic fibrosis (CF) is
now recognized as a single disease whose diverse symptoms stem from the wide tissue …

Structure, gating, and regulation of the CFTR anion channel

L Csanády, P Vergani, DC Gadsby - Physiological reviews, 2019 - journals.physiology.org
The cystic fibrosis transmembrane conductance regulator (CFTR) belongs to the ATP
binding cassette (ABC) transporter superfamily but functions as an anion channel crucial for …

Molecular structure of the human CFTR ion channel

F Liu, Z Zhang, L Csanády, DC Gadsby, J Chen - Cell, 2017 - cell.com
The cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-binding
cassette (ABC) transporter that uniquely functions as an ion channel. Here, we present a 3.9 …

Structure and function of the CFTR chloride channel

DN Sheppard, MJ Welsh - Physiological reviews, 1999 - journals.physiology.org
Sheppard, David N., and Michael J. Welsh. Structure and Function of the CFTR Chloride
Channel. Physiol. Rev. 79, Suppl.: S23–S45, 1999.—The cystic fibrosis transmembrane …

The ABC protein turned chloride channel whose failure causes cystic fibrosis

DC Gadsby, P Vergani, L Csanády - Nature, 2006 - nature.com
CFTR chloride channels are encoded by the gene mutated in patients with cystic fibrosis.
These channels belong to the superfamily of ABC transporter ATPases. ATP-driven …

Electrolyte transport in the mammalian colon: mechanisms and implications for disease

K Kunzelmann, M Mall - Physiological reviews, 2002 - journals.physiology.org
The colonic epithelium has both absorptive and secretory functions. The transport is
characterized by a net absorption of NaCl, short-chain fatty acids (SCFA), and water …

Chloride secretion by the intestinal epithelium: molecular basis and regulatory aspects

KE Barrett, SJ Keely - Annual review of physiology, 2000 - annualreviews.org
▪ Abstract Chloride secretion is the major determinant of mucosal hydration thoughout the
gastrointestinal tract, and chloride transport is also pivotal in the regulation of fluid secretion …

Physiological substrates of cAMP-dependent protein kinase

JB Shabb - Chemical reviews, 2001 - ACS Publications
The purpose of this review is to provide an account of the physiological substrates of cAMP-
dependent protein kinase (PKA). Discussion is limited to just over 100 unique gene products …

Structure of nucleotide‐binding domain 1 of the cystic fibrosis transmembrane conductance regulator

HA Lewis, SG Buchanan, SK Burley, K Conners… - The EMBO …, 2004 - embopress.org
Cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP‐binding cassette
(ABC) transporter that functions as a chloride channel. Nucleotide‐binding domain 1 …

Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis

DC Gadsby, AC Nairn - Physiological reviews, 1999 - journals.physiology.org
Gadsby, David C., and Angus C. Nairn. Control of CTFR Channel Gating by Phosphorylation
and Nucleotide Hydrolysis. Physiol. Rev. 79, Suppl.: S77–S107, 1999.—The cystic fibrosis …