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PMID: 22496330
Trinh NT, Bardou O, Prive A, Maille E, Adam D, Lingee S, Ferraro P, Desrosiers MY, Coraux C, Brochiero E
Improvement of defective cystic fibrosis airway epithelial wound repair after CFTR rescue.
Eur Respir J. 2012 Apr 10.,
[PubMed]
Sentences
No.
Mutations
Sentence
Comment
33
ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 22496330:33:198
status:
NEW
view ABCC7 p.Trp1282* details
ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 22496330:33:202
status:
NEW
view ABCC7 p.Trp1282* details
Cell culture Several non-CF and CF cell lines have been used: NuLi-1 (non-CF) and CuFi-1 (homozygous F508/F508) (gifted by Dr. J. Zabner, University of Iowa, [20]), IB3 (ΔF508/
W1282X, AT
CC) and S9 (wt-CFTR genetically repaired CF IB3 cells, ATCC), CFBE41o- transduced with wt-CFTR (CFBE-wt) or F508-CFTR (CFBE-F508) [21] (kindly provided by Dr. J. Hanrahan, McGill University).
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35
ABCC7 p.Leu206Trp
X
ABCC7 p.Leu206Trp 22496330:35:389
status:
NEW
view ABCC7 p.Leu206Trp details
ABCC7 p.Leu206Trp
X
ABCC7 p.Leu206Trp 22496330:35:393
status:
NEW
view ABCC7 p.Leu206Trp details
Primary human bronchial cells were isolated from CF homozygous F508/F508 lung transplants (median age 31 years, mean FEV1 23.6±2.1%) and non-CF donors, while primary human nasal cells were recovered after polypectomy procedures from non-CF and CF patients (with various genotypes, i.e. 2 homozygous F508/F508, 1 F508/A555E and 1 C621+1G
T/P-L206W
, median age 16 years, mean FEV1 of 81.6±14.2%), according to ethically-approved protocols.
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