PMID: 21525008

Suaud L, Miller K, Alvey L, Yan W, Robay A, Kebler C, Kreindler JL, Guttentag S, Hubbard MJ, Rubenstein RC
ERp29 regulates DeltaF508 and wild-type cystic fibrosis transmembrane conductance regulator (CFTR) trafficking to the plasma membrane in cystic fibrosis (CF) and non-CF epithelial cells.
J Biol Chem. 2011 Jun 17;286(24):21239-53. Epub 2011 Apr 27., 2011-06-17 [PubMed]
Sentences
No. Mutations Sentence Comment
17 ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 21525008:17:191
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 4-8 h after exposure and restores CFTR function at the plasma membrane without altering CFTR mRNA expression (16). Login to comment 48 ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 21525008:48:87
status: NEW
view ABCC7 p.Trp1282* details
 EXPERIMENTAL PROCEDURES Cell Culture-IB3-1 CF epithelial cells (genotype ⌬F508/ W1282X) (68) were cultured as described previously (4, 18). Login to comment