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. 2018 Jan 31;8:234. doi: 10.3389/fgene.2017.00234

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Copyright © 2018 Xiao and Zhou.

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 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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Three models of ZIP13's action proposed three entirely different etiology for SCD-EDS. (1) SCD-EDS is suggested to arise from increased zinc accumulation in the ER/Golgi. ZIP13 imports zinc from ER/Golgi to cytosol, so the absence of ZIP13 results in zinc accumulation in ER/Golgi. The accumulated zinc competes with iron for binding to LH and PH4, leading to disrupted biosynthesis of collagens and SCD-EDS. (2) SCD-EDS is due to zinc deficiency in the ER/Golgi. ZIP13 is responsible for zinc releasing from vesicular stores for use in the secretory pathway, so ZIP13 loss of function leads to general ER dysfunction and the pathogenesis of SCD-EDS. (3) SCD-EDS is caused by iron deficiency in the ER/Golgi. ZIP13 exports iron from cytosol into the secretory pathway, so ZIP13 loss of function results in iron deficiency in the ER/Golgi compartments, leading to collagen crosslinking defect and SCD-EDS.