Channelopathy Genes in Pulmonary Arterial Hypertension
Round 1
Reviewer 1 Report
The Authors of this article entitled “Channelopathy genes in pulmonary arterial hypertension” have written a concise review of the current knowledge of gene variants of ABCC8, ATP13A3, and KCNK3 in PAH.
Author Response
We appreciate the reviewer's comment. No further response was requested.
Reviewer 2 Report
The present manuscript reviews the present understanding of three main ion channels (ABCC8, ATP13A3, and KCNK3) and their roles in channellopathy associated with PAH. In addition the review also briefly summarizes the involvement of other channel genes (KCNA5, ABCC9). Overall, the authors did a commendable job to make the reader well-aligned with the present status of the concerned research area. I have enjoyed reading the manuscript.
I have a couple comments.
- Why mention channelopathy genes? The manuscript is not restricted to discussing the role of genes only. Protein data have presented and discussed in the manuscript.
- Why Kv1.5 dysfunction in PAH omitted? Ref: Pozeg, Z.I., Michelakis, E.D., McMurtry, S., Thébaud, B., Wu, X.C., Dyck, J.R.B. et al. (2003) In vivo gene transfer of the O2-sensitive potassium channel Kv1.5 reduces pulmonary hypertension and restores hypoxic pulmonary vasoconstriction in chronically hypoxic rats. Circulation 107, 2037–2044 https://doi.org/10.1161/01.CIR.0000062688.76508.B3.
- In PAH, increased TRPC6 expression promotes vasoconstriction and neomuscularisation of pulmonary arteries. The involvement of TRPC6 is not discussed in the present manuscript. Ref: Malczyk, M., Erb, A., Veith, C., Ghofrani, H.A., Schermuly, R.T., Gudermann, T. et al. (2017) The role of transient receptor potential channel 6 channels in the pulmonary vasculature. Front. Immunol. 8, 1–11 https://doi.org/10.3389/fimmu.2017.00707
Author Response
Please see attachment.
Author Response File: Author Response.pdf
