Clioquinol

Publication Title: 
Proceedings of the National Academy of Sciences of the United States of America

In investigating the role of metal ions in the pathogenesis of Huntington's disease, we examined the effects of clioquinol, a metal-binding compound currently in clinical trials for Alzheimer's disease treatment, on mutant huntingtin-expressing cells. We found that PC12 cells expressing polyglutamine-expanded huntingtin exon 1 accumulated less mutant protein and showed decreased cell death when treated with clioquinol. This effect was polyglutamine-length-specific and did not alter mRNA levels or protein degradation rates.

Author(s): 
Nguyen, Trent
Hamby, Aaron
Massa, Stephen M.
Publication Title: 
PloS One

BACKGROUND: Zinc deficiency due to poor nutrition or genetic mutations in zinc transporters is a global health problem and approaches to providing effective dietary zinc supplementation while avoiding potential toxic side effects are needed. METHODS/PRINCIPAL FINDINGS: Conditional knockout of the intestinal zinc transporter Zip4 (Slc39a4) in mice creates a model of the lethal human genetic disease acrodermatitis enteropathica (AE).

Author(s): 
Geiser, Jim
De Lisle, Robert C.
Finkelstein, David
Adlard, Paul A.
Bush, Ashley I.
Andrews, Glen K.
Publication Title: 
Chinese Medical Journal
Author(s): 
Katahira, K.
Kobayshi, T.
Higashida, T.
Kamae, S.
Nakajima, Y.
Fujimoto, S.
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