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Addendum

Spondylocheirodysplastic Ehlers-Danlos syndrome (SCD-EDS) and the mutant zinc transporter ZIP13

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Article: e974982 | Received 21 Aug 2014, Accepted 06 Oct 2014, Published online: 01 Dec 2014

Figures & data

Figure 1. Schematic working mechanism of a “corrector” and a “potentiator” for a pathogenic mutant form of ZIP13. (A) ZIP13 “corrector” inhibits the coordinated actions of cofactors including the identified molecules, VCP and HSP90, involved in the unfolding and transport of mutant ZIP13 to the proteasome. As a result, the “corrector” causes an effective amount of functional ZIP13 protein to accumulate. (B) ZIP13 “potentiator” alters the equilibrium toward the active form, improving the intracellular Zn homeostasis by increasing Zn influx.

Figure 1. Schematic working mechanism of a “corrector” and a “potentiator” for a pathogenic mutant form of ZIP13. (A) ZIP13 “corrector” inhibits the coordinated actions of cofactors including the identified molecules, VCP and HSP90, involved in the unfolding and transport of mutant ZIP13 to the proteasome. As a result, the “corrector” causes an effective amount of functional ZIP13 protein to accumulate. (B) ZIP13 “potentiator” alters the equilibrium toward the active form, improving the intracellular Zn homeostasis by increasing Zn influx.