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...

Deskribapen osoa

Gorde:
Xehetasun bibliografikoak
Argitaratua izan da:J Clin Invest
Egile Nagusiak: Rubenstein, R C, Egan, M E, Zeitlin, P L
Formatua: Artigo
Hizkuntza:Inglês
Argitaratua: American Society for Clinical Investigation 1997
Gaiak:
Research Article
Sarrera elektronikoa:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC508446/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/9366560/
https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI119788
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