Intranuclear inclusions in subtypes of striatal neurons in Huntington's disease transgenic mice

被引:24
作者
Kosinski, CM
Cha, JH
Young, AB
Mangiarini, L
Bates, G
Schiefer, J
Schwarz, M
机构
[1] Rhein Westfal TH Aachen, United Hosp, Dept Neurol, D-52074 Aachen, Germany
[2] Harvard Univ, Sch Med, Boston, MA 02114 USA
[3] Massachusetts Gen Hosp, Dept Neurol, Boston, MA 02114 USA
[4] Univ London Kings Coll, Guys Hosp, GKT Sch Med, Div Med & Mol Genet, London SE1 9RT, England
关键词
basal ganglia; Calbindin-D28K; choline acetyltransferase; Huntington's disease; immunohistochemistry; mice; transgenic; nuclear inclusion; ubiquitin;
D O I
10.1097/00001756-199912160-00031
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
R6/2 transgenic mice express exon 1 of an abnormal human Huntington's disease (HD) gene and develop a neurological phenotype similar to HD. These mice develop ubiquitinated neuronal intranuclear inclusions (NII) which might play a central role in the pathophysiology of HD. We studied the distribution of NII in subpopulations of striatal neurons in 12-week-old R6/2 transgenic mice using fluorescent double label immunohistochemistry. We observed that most of the Calbindin-D28K positive projection neurons (89%) and the Parvalbumin positive interneurons (86%) showed ubiquitinated NII. In interneurons, however, which contain either choline acetyltransferase, neuronal nitric oxide synthase, or Calretinin, the frequency of NII was much lower (22%, 8%, 9%, respectively). Our data suggest that subpopulations of striatal neurons differ remarkably in their capability of forming ubiquitinated NII. Interneurons which are known to resist neurodegeneration in HD show less NII. (C) 1999 Lippincott Williams & Wilkins.
引用
收藏
页码:3891 / 3896
页数:6
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