ABCMdb

PMID: 18574003

Roque T, Boncoeur E, Saint-Criq V, Bonvin E, Clement A, Tabary O, Jacquot J
Proinflammatory effect of sodium 4-phenylbutyrate in deltaF508-cystic fibrosis transmembrane conductance regulator lung epithelial cells: involvement of extracellular signal-regulated protein kinase 1/2 and c-Jun-NH2-terminal kinase signaling.
J Pharmacol Exp Ther. 2008 Sep;326(3):949-56. Epub 2008 Jun 23., [PubMed]

Sentences

No. Mutations Sentence Comment

8 ABCC7 p.Trp1282* Sodium 4-phenylbutyrate (4-PBA) restores chloride conductance by promoting trafficking of mature ⌬F508-CFTR to the cell membrane in the IB3-1 lung epithelial cell line (expressing the heterozygous ⌬F508/ W1282X mutation), the CFBE41o- lung epithelial cell line (expressing the homozygous ⌬F508/⌬F508 mutation), and This work was supported in part by grants from Institut National de la Sante´ et de la Recherche Me´dicale, the French Cystic Fibrosis Foundation Vaincre La Mucoviscidose (France) and Universite´ Pierre et Marie-Curie-Paris 06 (Grant BQR 2007). Login to comment 40 ABCC7 p.Trp1282* IB3-1 was a bronchial epithelial cell line derived from a CF patient (CFTR genotype ⌬F508/W1282X), and it was purchased from American Type Culture Collection (LGC Promochem SARL, Strasbourg, France). Login to comment 95 ABCC7 p.Trp1282* Results The effect of 4-PBA was investigated on two cultured CFBE41o- and IB3-1 lung epithelial cell lines (expressing the homozygous ⌬F508/⌬F508 mutation and heterozygous ⌬F508/W1282X, respectively). Login to comment