[HTML][HTML] Mutant (δF508) Cystic Fibrosis Transmembrane Conductance Regulator Cl− Channel Is Functional When Retained in Endoplasmic Reticulum of Mammalian …
EA Pasyk, JK Foskett - Journal of Biological Chemistry, 1995 - ASBMB
Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis
transmembrane conductance regulator (CFTR), a plasma membrane-localized chloride
channel. Some mutations in CFTR, including one which affects most patients (ΔF508-CFTR),
prevent CFTR from exiting the endoplasmic reticulum (ER) where it is synthesized. To
examine whether normal and mutant CFTRs function as chloride channels when they reside
in the ER, the patch clamp technique was used to measure currents in the outer membrane …
transmembrane conductance regulator (CFTR), a plasma membrane-localized chloride
channel. Some mutations in CFTR, including one which affects most patients (ΔF508-CFTR),
prevent CFTR from exiting the endoplasmic reticulum (ER) where it is synthesized. To
examine whether normal and mutant CFTRs function as chloride channels when they reside
in the ER, the patch clamp technique was used to measure currents in the outer membrane …