PMID: 22069317

Suaud L, Miller K, Panichelli AE, Randell RL, Marando CM, Rubenstein RC
4-Phenylbutyrate stimulates Hsp70 expression through the Elp2 component of elongator and STAT-3 in cystic fibrosis epithelial cells.
J Biol Chem. 2011 Dec 30;286(52):45083-92. Epub 2011 Nov 8., [PubMed]
Sentences
No. Mutations Sentence Comment
21 ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 22069317:21:191
status: NEW
view ABCC7 p.Trp1282* details
 Sodium 4-phenylbutyrate (4PBA) improves ⌬F508-CFTR intracellular trafficking in CF epithelial cells such as the IB3-1 CF human bronchiolar epithelial cell line (genotype ⌬F508/ W1282X) as early as 4 h after exposure, and restores CFTR function at the plasma membrane without altering CFTR mRNA expression (16). Login to comment