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PMID: 11491179
Rodgers HC, Knox AJ
Pharmacological treatment of the biochemical defect in cystic fibrosis airways.
Eur Respir J. 2001 Jun;17(6):1314-21.,
[PubMed]
Sentences
No.
Mutations
Sentence
Comment
48
ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 11491179:48:69
status:
NEW
view ABCC7 p.Trp1282* details
Forskolin-induced chloride secretion in IB3-1 cells expressing DF508/
W1282X
, and in primary human nasal epithelium, was increased by 4-PBA [13].
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60
ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 11491179:60:81
status:
NEW
view ABCC7 p.Trp1282* details
Similar results were seen in the human airway cell line IB3 derived from a DF508/
W1282X
CF patient [20].
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105
ABCC7 p.Trp1282*
X
ABCC7 p.Trp1282* 11491179:105:28
status:
NEW
view ABCC7 p.Trp1282* details
IB3 cells, heterozygous for
W1282X
treated with G418 or gentamicin, showed increased cAMP mediated current, although possibly via the ORCC rather than CFTR [42].
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