Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity
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...
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doaj-447d50972c724d6abbfdbe430156dea92020-11-24T22:57:50ZengMDPI AGBrain Sciences2076-34252017-06-01766310.3390/brainsci7060063brainsci7060063Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus NeurotoxicityRyoma Morigaki0Satoshi Goto1Parkinson’s Disease and Dystonia Research Center, Tokushima University Hospital, Tokushima University, Tokushima 770-8503, JapanParkinson’s Disease and Dystonia Research Center, Tokushima University Hospital, Tokushima University, Tokushima 770-8503, JapanHuntington’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.http://www.mdpi.com/2076-3425/7/6/63Huntington’s diseasehuntingtinstriatummedium spiny neuronpathophysiologystriosomematrix |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Ryoma Morigaki Satoshi Goto |
spellingShingle |
Ryoma Morigaki Satoshi Goto Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity Brain Sciences Huntington’s disease huntingtin striatum medium spiny neuron pathophysiology striosome matrix |
author_facet |
Ryoma Morigaki Satoshi Goto |
author_sort |
Ryoma Morigaki |
title |
Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity |
title_short |
Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity |
title_full |
Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity |
title_fullStr |
Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity |
title_full_unstemmed |
Striatal Vulnerability in Huntington’s Disease: Neuroprotection Versus Neurotoxicity |
title_sort |
striatal vulnerability in huntington’s disease: neuroprotection versus neurotoxicity |
publisher |
MDPI AG |
series |
Brain Sciences |
issn |
2076-3425 |
publishDate |
2017-06-01 |
description |
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. |
topic |
Huntington’s disease huntingtin striatum medium spiny neuron pathophysiology striosome matrix |
url |
http://www.mdpi.com/2076-3425/7/6/63 |
work_keys_str_mv |
AT ryomamorigaki striatalvulnerabilityinhuntingtonsdiseaseneuroprotectionversusneurotoxicity AT satoshigoto striatalvulnerabilityinhuntingtonsdiseaseneuroprotectionversusneurotoxicity |
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1725649035460608000 |