首页 | 本学科首页   官方微博 | 高级检索  
     


Mutant huntingtin activates Nrf2-responsive genes and impairs dopamine synthesis in a PC12 model of Huntington's disease
Authors:Willeke MC van Roon-Mom  Barry A Pepers  Peter AC 't Hoen  Carola ACM Verwijmeren  Johan T den Dunnen  Josephine C Dorsman  GertJan B van Ommen
Affiliation:(1) Center for Human and Clinical Genetics, Albinusdreef 2, 2333ZC Leiden, the Netherlands;(2) Department of Neurology, , Albinusdreef 2, 2333ZC Leiden, the Netherlands;(3) Leiden Genome Technology Center, Leiden University Medical Center, Albinusdreef 2, 2333ZC Leiden, the Netherlands;(4) Department of Clinical Genetics, Vrije Universiteit Medical Center, Van der Boechorststraat 7, 1081, BT, Amsterdam, the Netherlands
Abstract:

Background  

Huntington's disease is a progressive autosomal dominant neurodegenerative disorder that is caused by a CAG repeat expansion in the HD or Huntington's disease gene. Although micro array studies on patient and animal tissue provide valuable information, the primary effect of mutant huntingtin will inevitably be masked by secondary processes in advanced stages of the disease. Thus, cell models are instrumental to study early, direct effects of mutant huntingtin. mRNA changes were studied in an inducible PC12 model of Huntington's disease, before and after aggregates became visible, to identify groups of genes that could play a role in the early pathology of Huntington's disease.
Keywords:
本文献已被 SpringerLink 等数据库收录!
设为首页 | 免责声明 | 关于勤云 | 加入收藏

Copyright©北京勤云科技发展有限公司  京ICP备09084417号