Abstract：
: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 phenotypes of the Drosophila Nna1 ortholog (NnaD) with proteomics analysis of pcd mice. Reduced NnaD function yielded larval lethality, with survivors displaying phenotypes that mirror disease in pcd. Quantitative proteomics revealed expression alterations for glycolytic and oxidative phosphorylation enzymes. Nna proteins localize to mitochondria, loss of NnaD/Nna1 produces mitochondrial abnormalities, and pcd mice display altered proteolytic processing of Nna1 interacting proteins. Our studies indicate that Nna1 loss of function results in altered bioenergetics and mitochondrial dysfunction.
journal_name
Neuronjournal_title
Neuronauthors
Chakrabarti L,Zahra R,Jackson SM,Kazemi-Esfarjani P,Sopher BL,Mason AG,Toneff T,Ryu S,Shaffer S,Kansy JW,Eng J,Merrihew G,MacCoss MJ,Murphy A,Goodlett DR,Hook V,Bennett CL,Pallanck LJ,La Spada ARdoi
10.1016/j.neuron.2010.05.024subject
Has Abstractpub_date
2010-06-24 00:00:00pages
835-47issue
6eissn
0896-6273issn
1097-4199pii
S0896-6273(10)00420-4journal_volume
66pub_type
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