Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

By Motomasa Tanaka, Yoko Machida, Sanyong Niu, T. Ikeda, N. Jana, H. Doi, M. Kurosawa, Munenori Nekooki, N. Nukina. Nature Medicine, 2004

758 citations recorded by the source corpus.

Subfields: Cellular and Molecular Neuroscience, Molecular Biology, Neurology

View Paper: https://www.semanticscholar.org/paper/ca9c330cad3498cfbd1423f79c4f60bab67afd17

How researchers see this paper

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In Cellular and Molecular Neuroscience

1. C. Zuccato
2. E. Cattaneo
3. M. Valenza

In Molecular Biology

1. Jennifer L. Wacker
2. M. Valenza
3. E. Cattaneo
4. C. Zuccato
5. P. Muchowski

In Neurology

1. Jennifer L. Wacker
2. P. Muchowski

Citations in the corpus

• Effect of trehalose on protein structure (2008, background): “Disaccharides emerge as successful candidates with, perhaps not surprisingly, trehalose surfacing as the one which causes the most significant reduction in aggregation in vitro.(42) Other disaccha-”
• Molecular mechanisms and potential therapeutical targets in Huntington's disease. (2010, background): “In 2004, Nobuyuki Nukina and collaborators at the Riken Brain Science Institute in Japan (565) used an in vitro aggregate formation assay with myoglobin containing an expanded polyglutamine tract as target molecule and discovered that the disaccharide trehalose prevented nuclear inclusion formation,”
• Autophagy and Neurodegeneration: Pathogenic Mechanisms and Therapeutic Opportunities. (2017, neutral): “…FTD, HD, SCA17, PrP, ALS, and OPMD (Aguib et al., 2009; Castillo et al., 2013; Chen et al., 2015; Davies et al., 2006; Du et al., 2013; Li et al., 2015; Rodriguez-Navarro et al., 2010; Schaeffer and Goedert, 2012; Schaeffer et al., 2012; Tanaka et al., 2004; Tanji et al., 2015; Zhang et al., 2014).”
• Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death (2004, result): “Our results support the hypothesis that manipulations that improve survival and decrease IB formation (for example, certain aggregation inhibitors) might do so by interfering with the formation of toxic diffuse Htt species or with their ability to act on critical intracellular cellular target”
• Modulation of neurodegeneration by molecular chaperones (2005, background): “Trehalose was identified in an in vitro screen for inhibitors of polyQ aggregation, and its efficacy was subsequently evaluated in the R6/2 transgenic model of H”