PMID: 23346057

Villella VR, Esposito S, Bruscia EM, Maiuri MC, Raia V, Kroemer G, Maiuri L
Targeting the Intracellular Environment in Cystic Fibrosis: Restoring Autophagy as a Novel Strategy to Circumvent the CFTR Defect.
Front Pharmacol. 2013 Jan 21;4:1. doi: 10.3389/fphar.2013.00001. eCollection 2013., [PubMed]
Sentences
No. Mutations Sentence Comment
122 ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 23346057:122:304
status: NEW
view ABCC7 p.Trp1282* details
 Recently, we have reported that overexpression of BECN1, administration of cystamine, or depletion of SQSTM1 by RNA interference, can favor the trafficking of F508del-CFTR protein to the epithelial cell surface in vitro in CF epithelial cell lines (CFBE41o- or IB3-1, carrying F508del/F508del or F508del/W1282X CFTR,respectively),ex vivo in nasal polyp biopsies from CF patients, and in vivo in CftrF508del mice. Login to comment