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Huntingtin-Associated Proteins
2001Huntington’s disease (HD), with its writhing dancelike movements (chorea) and cardinal loss of neurons in the striatum (1), is the result of an unstable expanded CAG trinucleotide repeat that lengthens a variable glutamine tract in a novel protein called huntingtin (HD) (2).
Lucius A. Passani +2 more
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Characterization of neuron-specific huntingtin aggregates in human huntingtin knock-in mice
Neuroscience Research, 2007Huntington's disease (HD) is caused by a mutation causing expanded polyglutamine tracts in the N-terminal fragment of huntingtin. A pathological hallmark of HD is the formation of aggregates in the striatal neurons. Here we report that ageing human huntingtin knock-in mice expressing mutant human huntingtin contained neuronal huntingtin aggregates, as ...
Hisahide Takahashi +9 more
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Huntingtin facilitates selective autophagy
Nature Cell Biology, 2015Selective autophagy is essential for maintaining cellular homeostasis under different growth conditions. Huntingtin, mutated versions of which have been implicated in Huntington disease, is now shown to act as a scaffold protein that couples the induction of autophagy and the selective recruitment of cargo into autophagosomes.
Zvulun Elazar +2 more
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Huntingtin's critical cleavage
Nature Neuroscience, 2006The early pathogenic events leading to neurodegeneration in Huntington disease are not clear. A recent paper shows that mutating a caspase-6 cleavage site in the huntingtin protein is sufficient to prevent pathogenesis.
Huda Y. Zoghbi, John D. Fryer
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Hunting for the effects of huntingtin
Science, 2014Neurodegeneration Huntington's disease (HD) is associated with a mutant form of the protein huntingtin (Htt). HD-associated symptoms are alleviated by inhibition of the kinase mTOR, which activates protein synthesis when amino acids are plentiful. In mouse striatal neurons, Pryor et al.
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Huntington Disease and the Huntingtin Protein
2012Huntington disease (HD) is a devastating neurodegenerative disease that derives from CAG repeat expansion in the huntingtin gene. The clinical syndrome consists of progressive personality changes, movement disorder, and dementia and can develop in children and adults.
Zhiqiang Zheng, Marc I. Diamond
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