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

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-phenylbutyrate (4PBA), will enable a g...

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Hauptverfasser: Rubenstein, R C, Egan, M E, Zeitlin, P L
Format: Artigo
Sprache:Inglês
Veröffentlicht: 1997
Schlagworte:
Research Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC508446/
https://ncbi.nlm.nih.gov/pubmed/9366560
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