Article
Impact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating.
The Journal of physiology - 1 Jun 2015
Cai Zhiwei, Palmai-Pallag Timea, Khuituan Pissared, Mutolo Michael J, Boinot Clément, Liu Beihui, Scott-Ward Toby S, Callebaut Isabelle, Harris Ann, Sheppard David N
Abstract excerpt
KEY POINTS: Malfunction of the cystic fibrosis transmembrane conductance regulator (CFTR), a gated pathway for chloride movement, causes the common life-shortening genetic disease cystic fibrosis (CF). Towards the development of a sheep model of CF, we have investigated the function of sheep CFTR. We found that sheep CFTR was noticeably more active than human CFTR, while the most common CF mutation, F508del, had...
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