Mechanisms of neurodegeneration in Huntington's disease
by
Gil JM, Rego AC.
Island Medical Program,
Division of Medical Sciences,
University of Victoria,
Victoria, BC, Canada.
Eur J Neurosci. 2008 Jun;27(11):2803-20.

ABSTRACT

Huntington's disease (HD) is caused by an expansion of cytosine-adenine-guanine (CAG) repeats in the huntingtin gene, which leads to neuronal loss in the striatum and cortex and to the appearance of neuronal intranuclear inclusions of mutant huntingtin. Huntingtin plays a role in protein trafficking, vesicle transport, postsynaptic signaling, transcriptional regulation, and apoptosis. Thus, a loss of function of the normal protein and a toxic gain of function of the mutant huntingtin contribute to the disruption of multiple intracellular pathways. Furthermore, excitotoxicity, dopamine toxicity, metabolic impairment, mitochondrial dysfunction, oxidative stress, apoptosis, and autophagy have been implicated in the progressive degeneration observed in HD. Nevertheless, despite the efforts of a multidisciplinary scientific community, there is no cure for this devastating neurodegenerative disorder. This review presents an overview of the mechanisms that may contribute for HD pathogenesis. Ultimately, a better understanding of these mechanisms will lead to the development of more effective therapeutic targets.

HD
Biohappiness
Genospirituality
Private eugenics
'Designer babies'
Personal genomics
Genetic enhancement
Ashkenazi intelligence
Eugenics before Galton
Scandanavian eugenics
The literature of eugenics
Human self-domestication
Germline genetic engineering
Preimplantation genetic diagnosis
A life without pain? Hedonists take note'
Francis Galton and contemporary eugenics
Gene therapy and performance enhancement
Huntington's disease (HD): huntingtin functions to potential treatments

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