[HTML][HTML] Hyposecretion, Not Hyperabsorption, Is the Basic Defect of Cystic Fibrosis Airway Glands*♦
NS Joo, T Irokawa, RC Robbins, JJ Wine - Journal of biological chemistry, 2006 - ASBMB
Human airways and glands express the anion channel cystic fibrosis transmembrane
conductance regulator, CFTR, and the epithelial Na+ channel, ENaC. Cystic fibrosis (CF)
airway glands fail to secrete mucus in response to vasoactive intestinal peptide or forskolin;
the failure was attributed to loss of CFTR-mediated anion and fluid secretion. Alternatively,
CF glands might secrete acinar fluid via CFTR-independent pathways, but the exit of mucus
from the glands could be blocked by hyperabsorption of fluid in the gland ducts. This could …
conductance regulator, CFTR, and the epithelial Na+ channel, ENaC. Cystic fibrosis (CF)
airway glands fail to secrete mucus in response to vasoactive intestinal peptide or forskolin;
the failure was attributed to loss of CFTR-mediated anion and fluid secretion. Alternatively,
CF glands might secrete acinar fluid via CFTR-independent pathways, but the exit of mucus
from the glands could be blocked by hyperabsorption of fluid in the gland ducts. This could …