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. 2018 May 11;19:94. doi: 10.1186/s12931-018-0800-5

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© The Author(s). 2018

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Fig. 6 — Pathways of urea cycle disturbance in PAH. PAH = pulmonary artery hypertension; AMP = adenosine monophosphate; EC = endothelial cell; NO = nitric oxide; NOS = nitric oxide synthase; SMC = smooth muscle cell