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. Author manuscript; available in PMC: 2020 Feb 1.
Published in final edited form as: Trends Biochem Sci. 2018 Nov 13;44(2):125–140. doi: 10.1016/j.tibs.2018.10.005

Figure 4.

Figure 4.

Liver perfusion experiments showing that mouse liver produces large amounts of AMP and PPi, but not in mice without functional ABCC6. The very low levels of ATP in the perfusates are contributed to the rapid conversion of any ATP released into the perfusion buffer into AMP and PPi. In PXE patients reduced ABCC6-mediated ATP release results in low plasma PPi concentrations.