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Brain Sci. 2017, 7(6), 63; doi:10.3390/brainsci7060063

Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity

1,2,3
and
1,2,*
1
Parkinson’s Disease and Dystonia Research Center, Tokushima University Hospital, Tokushima University, Tokushima 770-8503, Japan
2
Department of Neurodegenerative Disorders Research, Institute of Biomedical Sciences, Graduate School of Medical Sciences, Tokushima University, Tokushima 770-8503, Japan
3
Department of Neurosurgery, Institute of Biomedical Sciences, Graduate School of Medical Sciences, Tokushima University, Tokushima 770-8503, Japan
*
Author to whom correspondence should be addressed.
Academic Editor: Edamakanti Chandrakanth Reddy
Received: 2 May 2017 / Revised: 2 June 2017 / Accepted: 3 June 2017 / Published: 7 June 2017
(This article belongs to the Special Issue Polyglutamine (PolyQ) Disorders)
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Abstract

Huntington’s disease (HD) is an autosomal dominant neurodegenerative disease caused by the expansion of a CAG trinucleotide repeat encoding an abnormally long polyglutamine tract (PolyQ) in the huntingtin (Htt) protein. In HD, striking neuropathological changes occur in the striatum, including loss of medium spiny neurons and parvalbumin-expressing interneurons accompanied by neurodegeneration of the striosome and matrix compartments, leading to progressive impairment of reasoning, walking and speaking abilities. The precise cause of striatal pathology in HD is still unknown; however, accumulating clinical and experimental evidence suggests multiple plausible pathophysiological mechanisms underlying striatal neurodegeneration in HD. Here, we review and discuss the characteristic neurodegenerative patterns observed in the striatum of HD patients and consider the role of various huntingtin-related and striatum-enriched proteins in neurotoxicity and neuroprotection. View Full-Text
Keywords: Huntington’s disease; huntingtin; striatum; medium spiny neuron; pathophysiology; striosome; matrix Huntington’s disease; huntingtin; striatum; medium spiny neuron; pathophysiology; striosome; matrix
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Morigaki, R.; Goto, S. Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity. Brain Sci. 2017, 7, 63.

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