Cystic fibrosis since 1938

PB Davis - American journal of respiratory and critical care …, 2006 - atsjournals.org
Cystic fibrosis (CF) was distinguished from celiac disease in 1938. Then, it was a pathologic
diagnosis, life expectancy was approximately 6 months, and the autosomal recessive …

The genesis of cystic fibrosis lung disease

JJ Wine - The Journal of clinical investigation, 1999 - Am Soc Clin Investig
James M. Wilson, Editor ductance, and by analogy with the sweat duct (11, 12) this allows
salt levels in the ASL to remain at levels similar to those in plasma. The high salt in the ASL …

[HTML][HTML] The cystic fibrosis transmembrane conductance regulator: an intriguing protein with pleiotropic functions

A Vankeerberghen, H Cuppens, JJ Cassiman - Journal of Cystic Fibrosis, 2002 - Elsevier
Cystic fibrosis is a frequent autosomal recessive disorder that is caused by the
malfunctioning of a small chloride channel, the cystic fibrosis transmembrane conductance …

Cystic fibrosis: exploiting its genetic basis in the hunt for new therapies

JL Kreindler - Pharmacology & therapeutics, 2010 - Elsevier
Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis
transmembrane conductance regulator (CFTR), an anion channel expressed in epithelial …

Strategies for the etiological therapy of cystic fibrosis

L Maiuri, V Raia, G Kroemer - Cell Death & Differentiation, 2017 - nature.com
Etiological therapies aim at repairing the underlying cause of cystic fibrosis (CF), which is
the functional defect of the cystic fibrosis transmembrane conductance regulator (CFTR) …

Cystic fibrosis: lessons from the sweat gland

PM Quinton - Physiology, 2007 - journals.physiology.org
Lessons from the sweat gland on cystic fibrosis (CF) began long before modern medicine
became a science. In European folklore, the curse that “a child that taste salty when kissed …

Novel pharmacologic therapies for cystic fibrosis

PL Zeitlin - The Journal of clinical investigation, 1999 - Am Soc Clin Investig
It has been nearly 10 years since the identification and cloning of the cystic fibrosis (CF)
gene was announced (1). Despite rapid advances in our understanding of the molecular …

Cystic fibrosis: a clinical view

C Castellani, BM Assael - Cellular and molecular life sciences, 2017 - Springer
Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on
chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas …

Aberrant CFTR-dependent HCO-3 transport in mutations associated with cystic fibrosis

JY Choi, D Muallem, K Kiselyov, MG Lee, PJ Thomas… - Nature, 2001 - nature.com
Cystic fibrosis (CF) is a disease caused by mutations in the cystic fibrosis transmembrane
conductance regulator (CFTR). Initially, Cl-conductance in the sweat duct was discovered to …

[HTML][HTML] Physiologic endpoints for clinical studies for cystic fibrosis

S Stanojevic, F Ratjen - Journal of Cystic fibrosis, 2016 - Elsevier
The cystic fibrosis (CF) drug development pipeline promises many exciting new treatments
for patients with CF, all which will require clinical studies to prove their benefits on CF lung …