Article
Mitochondrial dysfunction in NnaD mutant flies and Purkinje cell degeneration mice reveals a role for Nna proteins in neuronal bioenergetics.
Neuron - 24 Jun 2010
Chakrabarti Lisa, Zahra Rabaab, Jackson Stephen M, Kazemi-Esfarjani Parsa, Sopher Bryce L, Mason Amanda G, Toneff Thomas, Ryu Soyoung, Shaffer Scott, Kansy Janice W, Eng Jeremiah, Merrihew Gennifer, MacCoss Michael J, Murphy Anne, Goodlett David R, Hook Vivian, Bennett Craig L, Pallanck Leo J, La Spada Albert R
Abstract excerpt
The Purkinje cell degeneration (pcd) mouse is a recessive model of neurodegeneration, involving cerebellum and retina. Purkinje cell death in pcd is dramatic, as >99% of Purkinje neurons are lost in 3 weeks. Loss of function of Nna1 causes pcd, and Nna1 is a highly conserved zinc carboxypeptidase. To determine the basis of pcd, we implemented a two-pronged approach, combining characterization of loss-of-function...
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