Putting proteins in their place: palmitoylation in Huntington disease and other neuropsychiatric diseases

FB Young, SL Butland, SS Sanders, LM Sutton… - Progress in …, 2012 - Elsevier
Post-translational modification of proteins by the lipid palmitate is critical for protein
localization and function. Palmitoylation is regulated by the opposing enzymes palmitoyl …

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 …

Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and …

MA Pouladi, LM Stanek, Y Xie… - Human molecular …, 2012 - academic.oup.com
The development of animal models of Huntington disease (HD) has enabled studies that
help define the molecular aberrations underlying the disease. The BACHD and YAC128 …

Functional differences between direct and indirect striatal output pathways in Huntington's disease

L Galvan, VM André, EA Wang… - Journal of …, 2012 - content.iospress.com
There is morphological evidence for differential alterations in striatal medium-sized spiny
neurons (MSNs) giving rise to the direct and indirect output pathways in Huntington's …

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 …

Selective cognitive impairment in the YAC128 Huntington's disease mouse

SP Brooks, N Janghra, GV Higgs, Z Bayram-Weston… - Brain research …, 2012 - Elsevier
People with HD have a demonstrated early extra-dimensional set-shifting deficit. In the
present study, we use a novel water T-maze set-shifting procedure and demonstrate its …

The group 2 metabotropic glutamate receptor agonist LY379268 rescues neuronal, neurochemical and motor abnormalities in R6/2 Huntington's disease mice

A Reiner, DC Lafferty, HB Wang, N Del Mar… - Neurobiology of …, 2012 - Elsevier
Excitotoxic injury to striatum by dysfunctional cortical input or aberrant glutamate uptake may
contribute to Huntington's disease (HD) pathogenesis. Since corticostriatal terminals …

Light and electron microscopic characterization of the evolution of cellular pathology in YAC128 Huntington's disease transgenic mice

Z Bayram-Weston, L Jones, SB Dunnett… - Brain research bulletin, 2012 - Elsevier
Huntington's disease (HD) is a progressive neurodegenerative disease caused by the
insertion of an expanded polyglutamine sequence within the huntingtin protein. This …

Protein misfolding detected early in pathogenesis of transgenic mouse model of Huntington disease using amyloid seeding assay

S Gupta, DW Colby - Journal of Biological Chemistry, 2012 - ASBMB
Huntington disease (HD) is one of several fatal neurodegenerative disorders associated
with misfolded proteins. Here, we report a novel method for the sensitive detection of …

Light and electron microscopic characterization of the evolution of cellular pathology in the R6/1 Huntington's disease transgenic mice

Z Bayram-Weston, L Jones, SB Dunnett… - Brain research bulletin, 2012 - Elsevier
Huntington's disease (HD) is an inherited neurodegenerative disorder caused by an
expansion of CAG repeats in the Htt gene. Examination of the post-mortem brains of HD …