In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.

Other literature type English OPEN
Rubenstein, R C; Egan, M E; Zeitlin, P L;
  • Related identifiers: pmc: PMC508446
  • Subject: Research Article
    mesheuropmc: respiratory system | congenital, hereditary, and neonatal diseases and abnormalities | respiratory tract diseases | digestive system diseases

The most common cystic fibrosis transmembrane conductance regulator mutation, delta F508-CFTR, is a partially functional chloride channel that is retained in the endoplasmic reticulum and degraded. We hypothesize that a known transcriptional regulator, sodium 4-phenylbu... View more
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