Publication: Cellular and molecular mechanisms involved in the selective vulnerability of striatal projection neurons in Huntington`s disease
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Date
2006
Authors
Pérez-Navarro, E. ; Canals, J.M. ; Ginés, S. ; Alberch, J.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Neurodegenerative disorders affecting the
central nervous system, such as Alzheimer’s disease,
Parkinson’s disease, Huntington’s chorea (HD) and
amyotrophic lateral sclerosis are characterized by the
loss of selected neuronal populations. Another striking
feature shared by these diseases is the deposition of
proteinaceous inclusion bodies in the brain, which may
be intracytoplasmatic or intranuclear, or even
extracellular. However, the density and prevalence of
aggregates are not always directly related to
neurodegeneration. Although some of these diseases are
the result of mutations in known proteins, with HD a
clear example, the expression and location of the
affected protein do not explain the selective
neurodegeneration. Therefore, other intrinsic
mechanisms, characteristic of each neuronal population,
might be involved in the neurodegenerative process. In
this review we focus on several proposed mechanisms
such as excitotoxicity, mitochondrial dysfunction and
altered expression of trophic factors, which could
account for the pathogenesis of HD.
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