Mutant huntingtin activates Nrf2-responsive genes and impairs dopamine synthesis in a PC12 model of Huntington's disease |
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| 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 |
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| Institution: | (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 |
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| 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. |
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