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]. Login to comment
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]. Login to comment
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]. Login to comment