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. 2020 Dec 23;11:604580. doi: 10.3389/fphys.2020.604580

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Copyright © 2020 Silva, Duarte, Marson, Centeio, Doušová, Kunzelmann and Amaral.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Representative original tracings from Ussing chamber measurements of CFTR-mediated Cl^– secretion in rectal biopsies from 143 individuals with three distinctive patterns. Effects of cholinergic stimulation by carbachol (CCH, 100 μM, basolateral, blue box), cAMP-dependent by IBMX/Forskolin (I/F, 100 μM/2 μM, basolateral), and I/F/C combined on transepithelial voltage (V_te) represented by the red box, on rectal biopsies from: (A) non-CF individuals, (B) individuals with atypical CF, and (C) individuals with classical CF. The gray box represents the effect of the last I/F/C response. Experiments were performed in the presence of Amiloride (Amil, 20 μM, luminal) and/or Indomethacin (Indo, 10 μM, basolateral), as indicated above tracings.