ABCMdb

PMID: 20147297

Henderson MJ, Vij N, Zeitlin PL
Ubiquitin C-terminal hydrolase-L1 protects cystic fibrosis transmembrane conductance regulator from early stages of proteasomal degradation.
J Biol Chem. 2010 Apr 9;285(15):11314-25. Epub 2010 Feb 10., 2010-04-09 [PubMed]

Sentences

No. Mutations Sentence Comment

42 ABCC7 p.Trp1282* ABCC7 p.Trp1282* EXPERIMENTAL PROCEDURES Cell Lines and Culture-The CF bronchial epithelial cell line IB3-1 (⌬F508/W1282X; low level expression of ⌬F508-CFTR and no W1282X protein) (35), a CF tracheal epithelial cell line CFTE (⌬F508-homozygous) (36), and a CF line with wild type phenotype S9 (IB3-1 corrected by AAV-CFTR) (37) were maintained in LHC-8 medium with 10% fetal bovine serum, 100 units/ml penicillin, 100 ␮g/ml streptomycin, and 0.25 ␮g/ml amphotericin B at 37 °C in the presence of 5% CO2. Login to comment 86 ABCC7 p.Trp1282* ABCC7 p.Trp1282* Whole cell protein lysates (30 ␮g) from IB3-1 (⌬F508/W1282X), CFTE (⌬F508/⌬F508), and S9 (⌬F508/W1282X ϩ AAV-WT CFTR) were resolved by SDS-PAGE and immunoblotted with antibodies to UCH-L1 (upper panel), UCH-L3 (middle panel), and actin (lower panel). Login to comment 110 ABCC7 p.Trp1282* ABCC7 p.Trp1282* To validate these earlier observations, we performed Western blot analysis on cell lysates from IB3-1 (⌬F508/W1282X) and S9 (CF corrected - ⌬F508/W1282X ϩ AAV-WT-CFTR) cells and a second CF cell line homozygous for ⌬F508 (CFTE). Login to comment