Journal
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
Volume 97, Issue 12, Pages 6809-6814Publisher
NATL ACAD SCIENCES
DOI: 10.1073/pnas.120166397
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- NIMH NIH HHS [MH-40899, P01 MH040899] Funding Source: Medline
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In Huntington's disease (HD), mutation of huntingtin causes selective neurodegeneration of dopaminoceptive striatal medium spiny neurons. Transgenic: Ho model mice that express a portion of the disease-causing form of human huntingtin develop a behavioral phenotype that suggests dysfunction of dopaminergic neurotransmission, Here we show that presymtomatic mice have severe deficiencies in dopamine signaling in the striatum, These include selective reductions in total levels of dopamine- and cAMP-regulated phosphoprotein, MI 32 kDA (DARPP-32) and other dopamine-regulated phosphoprotein markers of medium spiny neurons, HD mice also show defects in dopamine-regulated ion channels and in the D-1 dopamine/DARPP-32 signaling cascade. These presymptomatic defects may contribute to Ho pathology.
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