Tuesday, November 11, 2014

Fly study relevant to Menkes and Wilson diseases

Hwang JE, de Bruyne M, Warr CG, Burke R. Copper overload and deficiency both adversely affect the central nervous system of Drosophila. Metallomics. 2014 Oct 17. PMID: 25322772.

From the abstract: "The human copper homeostasis disorders Menkes and Wilson disease both have severe neurological symptoms. Menkes is a copper deficiency disorder whereas Wilson disease patients suffer from copper toxicity, indicating that tight control of neuronal copper levels is essential for proper nervous system development and function. Here we examine the consequences of neuronal copper deficiency and excess in the Drosophila melanogaster nervous system, using targeted manipulation of the copper uptake genes Ctr1A and Ctr1B and efflux gene ATP7 in combination with altered dietary copper levels. ... We conclude that both copper overload and excess have detrimental effects on Drosophila neuronal function, reducing overall fly viability as well as impacting on a specific neuropeptide pathway."

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