Hypothalamic–endocrine aspects in Huntington's disease

Å Petersén, M Björkqvist - European Journal of Neuroscience, 2006 - Wiley Online Library
Huntington's disease (HD) is a hereditary and fatal disorder caused by an expanded CAG
triplet repeat in the HD gene, resulting in a mutant form of the protein huntingtin. Wild‐type …

Selective neuronal degeneration in Huntington's disease

CM Cowan, LA Raymond - Current topics in developmental biology, 2006 - Elsevier
Huntington's disease (HD) is a progressive neurodegenerative disorder that generally
begins in middle age with abnormalities of movement, cognition, personality, and mood …

Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin

RK Graham, Y Deng, EJ Slow, B Haigh, N Bissada… - Cell, 2006 - cell.com
Cleavage of huntingtin (htt) has been characterized in vitro, and accumulation of caspase
cleavage fragments represents an early pathological change in brains of Huntington's …

Palmitoylation of huntingtin by HIP14is essential for its trafficking and function

A Yanai, K Huang, R Kang, RR Singaraja… - Nature …, 2006 - nature.com
Post-translational modification by the lipid palmitate is crucial for the correct targeting and
function of many proteins. Here we show that huntingtin (htt) is normally palmitoylated at …

Huntington's disease: seeing the pathogenic process through a genetic lens

JF Gusella, ME MacDonald - Trends in biochemical sciences, 2006 - cell.com
Thirteen years ago, the culmination of genetic rather than biochemical strategies resulted in
the identification of the root cause of Huntington's disease: an expanded CAG trinucleotide …

CA150 expression delays striatal cell death in overexpression and knock-in conditions for mutant huntingtin neurotoxicity

M Arango, S Holbert, D Zala, E Brouillet… - Journal of …, 2006 - Soc Neuroscience
Transcriptional dysregulation caused by expanded polyglutamines (polyGlns) in huntingtin
(htt) may be central to cell-autonomous mechanisms for neuronal cell death in Huntington's …

Animal models of Huntington's disease: implications in uncovering pathogenic mechanisms and developing therapies1

L WANG, Z QIN - Acta pharmacologica sinica, 2006 - Wiley Online Library
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder, which is
caused by an abnormal expansion of Cytosine Adenine Guanine (CAG) trinucleotide repeat …

To be or not to be toxic: aggregations in Huntington and Alzheimer disease

EJ Slow, RK Graham, MR Hayden - Trends in Genetics, 2006 - cell.com
Insoluble aggregated proteins in Alzheimer disease and Huntington disease might not be
pathogenic. Human studies have poor correlations between aggregates and clinical disease …

Huntingtin's critical cleavage

JD Fryer, HY Zoghbi - Nature neuroscience, 2006 - nature.com
Huntingtin's critical cleavage | Nature Neuroscience Skip to main content Thank you for visiting
nature.com. You are using a browser version with limited support for CSS. To obtain the best …

[图书][B] Huntington's disease: Mechanisms of nuclear accumulation and toxicity of mutant Huntingtin

JC Cornett - 2006 - search.proquest.com
Huntington's disease (HD) is one of at least nine inherited neurodegenerative disorders
caused by polyglutamine (polyQ) repeat expansion in the associated disease proteins. In …