Molecular mechanisms and potential therapeutical targets in Huntington's disease

C Zuccato, M Valenza, E Cattaneo - Physiological reviews, 2010 - journals.physiology.org
Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG repeat
expansion in the gene encoding for huntingtin protein. A lot has been learned about this …

Early synaptic pathophysiology in neurodegeneration: insights from Huntington's disease

AJ Milnerwood, LA Raymond - Trends in neurosciences, 2010 - cell.com
Investigations of synaptic transmission and plasticity in mouse models of Huntington's
disease (HD) demonstrate neuronal dysfunction long before the onset of classical disease …

Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease

C Landles, K Sathasivam, A Weiss, B Woodman… - Journal of Biological …, 2010 - ASBMB
Huntingtin proteolysis has been implicated in the molecular pathogenesis of Huntington
disease (HD). Despite an intense effort, the identity of the pathogenic smallest N-terminal …

Neuroinflammation in Huntington's disease

T Möller - Journal of neural transmission, 2010 - Springer
Huntington's disease (HD) is a monogenic neurodegenerative disease characterized by
abnormal motor movements, personality changes and early death. In contrast to other …

Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease

K Sathasivam, A Lane, J Legleiter… - Human molecular …, 2010 - academic.oup.com
Huntington's disease (HD) is a late-onset neurodegenerative disorder that is characterized
neuropathologically by the presence of neuropil aggregates and nuclear inclusions …

Widespread decrease of type 1 cannabinoid receptor availability in Huntington disease in vivo

K Van Laere, C Casteels, I Dhollander… - Journal of Nuclear …, 2010 - Soc Nuclear Med
The type 1 cannabinoid receptor (CB1) is a crucial modulator of synaptic transmission in the
brain. Animal and postmortem human data suggest that mutant huntingtin represses CB1 …

Transcriptional changes in Huntington disease identified using genome-wide expression profiling and cross-platform analysis

K Becanovic, MA Pouladi, RS Lim… - Human molecular …, 2010 - academic.oup.com
Abstract Evaluation of transcriptional changes in the striatum may be an effective approach
to understanding the natural history of changes in expression contributing to the …

Changes in key hypothalamic neuropeptide populations in Huntington disease revealed by neuropathological analyses

S Gabery, K Murphy, K Schultz, CT Loy… - Acta …, 2010 - Springer
Huntington disease (HD) is a fatal neurodegenerative disorder caused by expansion of a
CAG repeat in the HD gene. Degeneration concentrating in the basal ganglia has been …

Calcium signaling and neurodegeneration

IB Bezprozvanny - Acta Naturae (англоязычная версия), 2010 - cyberleninka.ru
Neurodegenerative disorders, such as Alzheimer's disease (AD), Parkinson's disease (PD),
amyotrophic lateral sclerosis (ALS), Huntington's disease (HD), and spinocerebellar ataxias …

HIF prolyl hydroxylase inhibitors prevent neuronal death induced by mitochondrial toxins: therapeutic implications for Huntington's disease and Alzheimer's disease

Z Niatsetskaya, M Basso, RE Speer… - Antioxidants & redox …, 2010 - liebertpub.com
Mitochondrial dysfunction is a central feature of a number of acute and chronic
neurodegenerative conditions, but clinically approved therapeutic interventions are only just …