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  Huntington's Disease: from Molecular Basis to Therapeutic Advances.

Krobitsch, S., & Kazantsev, A. G. (2011). Huntington's Disease: from Molecular Basis to Therapeutic Advances. The International Journal of Biochemistry & Cell Biology., 43(1), 20-24. doi:10.1016/j.biocel.2010.10.014 |.

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Item Permalink: http://hdl.handle.net/11858/00-001M-0000-0010-77D5-9 Version Permalink: http://hdl.handle.net/11858/00-001M-0000-0010-77D6-7
Genre: Journal Article
Alternative Title : Int. J. Biochem. Cell Biol.

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 Creators:
Krobitsch, Sylvia1, Author              
Kazantsev, Aleksey G.2, Author
Affiliations:
1Neurodegenerative Disorders (Sylvia Krobitsch), Independent Junior Research Groups (OWL), Max Planck Institute for Molecular Genetics, Max Planck Society, ou_1479661              
2Max Planck Society, ou_persistent13              

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Free keywords: Neurodegeneration; Huntington's disease; Huntingtin; Neuroprotection
 Abstract: Huntington's disease is an autosomal dominant genetic neurodegenerative disorder, which is characterized by progressive motor dysfunction, emotional disturbances, dementia, and weight loss. The disease is caused by pathological CAG-triplet repeat extension(s), encoding polyglutamines, within the gene product, huntingtin. Huntingtin is ubiquitously expressed through the body and is a protein of uncertain molecular function(s). Mutant huntingtin, containing pathologically extended polyglutamines causes the earliest and most dramatic neuropathologic changes in the neostriatum and cerebral cortex. Extended polyglutamines confer structural conformational changes to huntingtin, which gains novel properties, resulting in aberrant interactions with multiple cellular components. The diverse and variable aberrations mediated by mutant huntingtin perturb many cellular functions essential for neuronal homeostasis and underlie pleiotropic mechanisms of Huntington's disease pathogenesis. The only approved drug for Huntington's disease is a symptomatic treatment, tetrabenazine; thus, novel neuroprotective strategies, slowing, blocking and possibly reversing disease progression, are vital for developing effective therapies.

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Language(s): eng - English
 Dates: 2011-01-01
 Publication Status: Published in print
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Title: The International Journal of Biochemistry & Cell Biology.
  Alternative Title : Int. J. Biochem. Cell Biol.
Source Genre: Journal
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Pages: - Volume / Issue: 43 (1) Sequence Number: - Start / End Page: 20 - 24 Identifier: ISSN: 1357-2725