[HTML][HTML] Antioxidants in Huntington's disease

A Johri, MF Beal - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2012 - Elsevier
Huntington's disease (HD) is a prototypical neurodegenerative disease in which there is
selective neuronal degeneration, which leads to progressive disability, manifesting itself as …

Huntington disease and the huntingtin protein

Z Zheng, MI Diamond - Progress in molecular biology and translational …, 2012 - Elsevier
Huntington disease (HD) is a devastating neurodegenerative disease that derives from CAG
repeat expansion in the huntingtin gene. The clinical syndrome consists of progressive …

[HTML][HTML] Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175

LB Menalled, AE Kudwa, S Miller, J Fitzpatrick… - PloS one, 2012 - journals.plos.org
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder
characterized by motor, cognitive and psychiatric manifestations. Since the mutation …

Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy

RL Vidal, A Figueroa, FA Court, P Thielen… - Human molecular …, 2012 - academic.oup.com
Mutations leading to expansion of a poly-glutamine track in Huntingtin (Htt) cause
Huntington's disease (HD). Signs of endoplasmic reticulum (ER) stress have been recently …

Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice

A Di Pardo, V Maglione, M Alpaugh… - Proceedings of the …, 2012 - National Acad Sciences
Huntington disease (HD) is a progressive neurodegenerative monogenic disorder caused
by expansion of a polyglutamine stretch in the huntingtin (Htt) protein. Mutant huntingtin …

[HTML][HTML] Improvement of neuropathology and transcriptional deficits in CAG 140 knock-in mice supports a beneficial effect of dietary curcumin in Huntington's disease

MA Hickey, C Zhu, V Medvedeva, RP Lerner… - Molecular …, 2012 - Springer
Backgound No disease modifying treatment currently exists for Huntington's disease (HD), a
fatal neurodegenerative disorder characterized by the formation of amyloid-like aggregates …

Modeling human neurodegenerative diseases in transgenic systems

MA Gama Sosa, R De Gasperi, GA Elder - Human genetics, 2012 - Springer
Transgenic systems are widely used to study the cellular and molecular basis of human
neurodegenerative diseases. A wide variety of model organisms have been utilized …

Increased brain tissue sodium concentration in Huntington's Disease—a sodium imaging study at 4 T

K Reetz, S Romanzetti, I Dogan, C Saß, CJ Werner… - Neuroimage, 2012 - Elsevier
The neuropathological hallmark of the autosomal dominantly inherited, neurodegenerative
disorder Huntington's disease is progressive striatal loss starting several years prior to …

[HTML][HTML] Msh2 Acts in Medium-Spiny Striatal Neurons as an Enhancer of CAG Instability and Mutant Huntingtin Phenotypes in Huntington's Disease Knock-In Mice

M Kovalenko, E Dragileva, J St. Claire, T Gillis… - 2012 - journals.plos.org
The CAG trinucleotide repeat mutation in the Huntington's disease gene (HTT) exhibits age-
dependent tissue-specific expansion that correlates with disease onset in patients …

[HTML][HTML] Mouse models of polyglutamine diseases: review and data table. Part I

M Figiel, WJ Szlachcic, PM Switonski, A Gabka… - Molecular …, 2012 - Springer
Polyglutamine (polyQ) disorders share many similarities, such as a common mutation type in
unrelated human causative genes, neurological character, and certain aspects of …