Differential vulnerability of neurons in Huntington's disease: The role of cell type-specific features
| Autor: | Gerardo Morfini, Ina Han, YiMei You, Jeffrey H. Kordower, Scott T. Brady |
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| Jazyk: | angličtina |
| Rok vydání: | 2010 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities Huntingtin Gene Expression Context (language use) Nerve Tissue Proteins Striatum Biology Biochemistry Axonal Transport Article Cellular and Molecular Neuroscience Huntington's disease mental disorders Huntingtin Protein medicine Humans Brain-derived neurotrophic factor Brain Chemistry Neurons Brain-Derived Neurotrophic Factor Brain Nuclear Proteins Polyglutamine tract medicine.disease nervous system diseases Mitochondria medicine.anatomical_structure Huntington Disease nervous system Cerebral cortex Mutation Nerve Degeneration Neuroscience Signal Transduction |
| Popis: | J. Neurochem. (2010) 113, 1073–1091. Abstract Abnormal expansion of a polyglutamine tract in huntingtin (Htt) protein results in Huntington’s disease (HD), an autosomal dominant neurodegenerative disorder involving progressive loss of motor and cognitive function. Contrasting with the ubiquitous tissue expression of polyglutamine-expanded Htt, HD pathology is characterized by the increased vulnerability of specific neuronal populations within the striatum and the cerebral cortex. Morphological, biochemical, and functional characteristics of neurons affected in HD that might render these cells more vulnerable to the toxic effects of polyglutamine-Htt are covered in this review. The differential vulnerability of neurons observed in HD is discussed in the context of various major pathogenic mechanisms proposed to date, and in line with evidence showing a ‘dying-back’ pattern of degeneration in affected neuronal populations. |
| Databáze: | OpenAIRE |
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