@article {1291,
	title = {MicroRNA pathways modulate polyglutamine-induced neurodegeneration.},
	journal = {Molecular Cell},
	volume = {24},
	year = {2006},
	month = {2006 Oct 6},
	pages = {157-63},
	abstract = {<p>Nine human neurodegenerative diseases are due to expansion of a CAG repeat- encoding glutamine within the open reading frame of the respective genes. Polyglutamine (polyQ) expansion confers dominant toxicity, resulting in neuronal degeneration. MicroRNAs (miRNAs) have been shown to modulate programmed cell death during development. To address whether miRNA pathways play a role in neurodegeneration, we tested whether genes critical for miRNA processing modulated toxicity induced by the spinocerebellar ataxia type 3 (SCA3) protein. These studies revealed a striking enhancement of polyQ toxicity upon reduction of miRNA processing in Drosophila and human cells. In parallel genetic screens, we identified the miRNA bantam (ban) as a potent modulator of both polyQ and tau toxicity in flies. Our studies suggest that ban functions downstream of toxicity of the SCA3 protein, to prevent degeneration. These findings indicate that miRNA pathways dramatically modulate polyQ- and tau-induced neurodegeneration, providing the foundation for new insight into therapeutics.</p>
},
	keywords = {Animals, Cyclins, DNA, Drosophila, Drosophila Proteins, Genetically Modified, Hela Cells, Humans, MicroRNAs, Nerve Tissue Proteins, Neurodegenerative Diseases, Neurons, Nuclear Proteins, Open Reading Frames, Peptides, Repressor Proteins, Retina, tau Proteins, Trinucleotide Repeat Expansion},
	issn = {1097-2765},
	doi = {10.1016/j.molcel.2006.07.030},
	author = {Bilen, Julide and Liu, Nan and Burnett, Barrington G and Pittman, Randall N and Bonini, Nancy M}
}
@article {1289,
	title = {A new role for microRNA pathways: modulation of degeneration induced by pathogenic human disease proteins.},
	journal = {Cell Cycle },
	volume = {5},
	year = {2006},
	month = {2006 Dec},
	pages = {2835-8},
	abstract = {<p>MicroRNAs (miRNAs) are small noncoding RNAs that regulate the expression of target transcript mRNAs. Many miRNAs have been defined, however their roles and the processes influenced by miRNA pathways are still being elucidated. A role for miRNAs in development and cancer has been described. We recently isolated the miRNA bantam (ban) in a genetic screen for modulators of pathogenicity of a human neurodegenerative disease model in Drosophila. These studies showed that upregulation of ban mitigates degeneration induced by the pathogenic polyglutamine (polyQ) protein Ataxin-3, which is mutated in the human polyglutamine disease spinocerebellar ataxia type 3 (SCA3). To address the broader role for miRNAs in neuroprotection, we also showed that loss of all miRNAs, by dicer mutation, dramatically enhances pathogenic polyQ protein toxicity in flies and in human HeLa cells. These studies suggest that miRNAs may be important for neuronal survival in the context of human neurodegenerative disease. These studies provide the foundation to define the miRNAs involved in neurodegenerative disease, and the biological pathways affected.</p>
},
	keywords = {Animal, Animals, Cyclins, Disease Models, Drosophila, Drosophila Proteins, Eye, Hela Cells, Humans, MicroRNAs, Nerve Degeneration, Neurodegenerative Diseases, Peptides, Trinucleotide Repeat Expansion},
	issn = {1551-4005},
	author = {Bilen, Julide and Liu, Nan and Bonini, Nancy M}
}
