Striatal Vulnerability in Huntington’s Disease : Neuroprotection Versus Neurotoxicity

• Morigaki, Ryoma

• Goto, Satoshi

抄録

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.

収録刊行物

• Brain Sciences

  Brain Sciences 7 (6), 63-, 2017-06-07

  MDPI

キーワード

• Huntington’s disease
• huntingtin
• striatum
• medium spiny neuron
• pathophysiology
• striosome
• matrix

詳細情報

• CRID: 1050565163715642496
• NII論文ID: 120006808636
• ISSN: 20763425
• DOI: 10.3390/brainsci7060063
• Web Site: http://repo.lib.tokushima-u.ac.jp/114299
• Web Site: http://www.mdpi.com/2076-3425/7/6/63/pdf
• 本文言語コード: en
• 資料種別: journal article
• データソース種別:
  • IRDB
  • Crossref
  • CiNii Articles
  • KAKEN