Thursday, December 15, 2016

Review highlights use of mouse, fly, and other model organisms to study Menkes disease

Lenartowicz M, Krzeptowski W, Lipiński P, Grzmil P, Starzyński R, Pierzchała O, Møller LB. Mottled Mice and Non-Mammalian Models of Menkes Disease. Front Mol Neurosci. 2015 Dec 18;8:72. PMID: 26732058; PMCID: PMC4684000.

From the abstract: "Menkes disease is a multi-systemic copper metabolism disorder caused by mutations in the X-linked ATP7A gene and characterized by progressive neurodegeneration and severe connective tissue defects. .... Mottled mutants closely recapitulate the Menkes phenotype and are invaluable for studying Cu-metabolism. They provide useful models for exploring and testing new forms of therapy in Menkes disease. Recently, non-mammalian models of Menkes disease, Drosophila melanogaster and Danio rerio mutants were used in experiments which would be technically difficult to carry out in mammals."

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