Mutant protein in Huntington disease is resistant to proteolysis in affected brain
Author:
Publisher
Springer Science and Business Media LLC
Subject
Genetics
Link
http://www.nature.com/articles/ng745z.pdf
Reference48 articles.
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3. Gutekunst, C.-A. et al. Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. J. Neurosci. 19, 2522–2534 (1999).
4. Li, H., Li, S.H., Johnston, H., Shelbourne, P.F. & Li, X.J. Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity. Nature Genet. 25, 385–389 (2000).
5. Ross, C.A. Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegeneration disease? Neuron 19, 1147–1150 (1997).
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