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. 2016 Nov 25;6:36541. doi: 10.1038/srep36541

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Copyright © 2016, The Author(s)

This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

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CPSI is the rate limiting step in the urea cycle, and it was found down-regulated in the plasma of CHD-PAH in our study. ASS, argininosuccinate synthase; ASL, argininosuccinate lyase; NOS, nitric oxide synthase; OTC, ornithine transcarbamylase. (The diagram is modified from Pearson DL. et al.²²).