SAP97-mediated rescue of NMDA receptor surface distribution in a neuronal model of Huntington's disease

Hippocampus ◽  
2018 ◽  
Vol 28 (10) ◽  
pp. 707-723 ◽  
Author(s):  
Wojciech Ambroziak ◽  
Chantelle Fourie ◽  
Johanna M. Montgomery
Keyword(s):  
Nmda Receptor ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuronal Model ◽  
Surface Distribution

NMDA receptor losses in putamen from patients with Huntington's disease

Science ◽  
1988 ◽  
Vol 241 (4868) ◽  
pp. 981-983 ◽  
Author(s):  
A. Young ◽  
J. Greenamyre ◽  
Z Hollingsworth ◽  
R Albin ◽  
C D'Amato ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Huntington's Disease ◽  
Huntington’S Disease

scholarly journals Early dysfunction and progressive degeneration of the subthalamic nucleus in mouse models of Huntington's disease

eLife ◽  
2016 ◽  
Vol 5 ◽  
Author(s):  
Jeremy F Atherton ◽  
Eileen L McIver ◽  
Matthew RM Mullen ◽  
David L Wokosin ◽  
D James Surmeier ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Mouse Models ◽  
Subthalamic Nucleus ◽  
Glutamate Uptake ◽  
Oxidant Stress ◽  
Katp Channels ◽  
Receptor Antagonism ◽  
Progressive Degeneration

The subthalamic nucleus (STN) is an element of cortico-basal ganglia-thalamo-cortical circuitry critical for action suppression. In Huntington's disease (HD) action suppression is impaired, resembling the effects of STN lesioning or inactivation. To explore this potential linkage, the STN was studied in BAC transgenic and Q175 knock-in mouse models of HD. At <2 and 6 months of age autonomous STN activity was impaired due to activation of KATP channels. STN neurons exhibited prolonged NMDA receptor-mediated synaptic currents, caused by a deficit in glutamate uptake, and elevated mitochondrial oxidant stress, which was ameliorated by NMDA receptor antagonism. STN activity was rescued by NMDA receptor antagonism or the break down of hydrogen peroxide. At 12 months of age approximately 30% of STN neurons had been lost, as in HD. Together, these data argue that dysfunction within the STN is an early feature of HD that may contribute to its expression and course.

scholarly journals Early Increase in Extrasynaptic NMDA Receptor Signaling and Expression Contributes to Phenotype Onset in Huntington's Disease Mice

Neuron ◽  
2010 ◽  
Vol 65 (2) ◽  
pp. 178-190 ◽  
Author(s):  
Austen J. Milnerwood ◽  
Clare M. Gladding ◽  
Mahmoud A. Pouladi ◽  
Alexandra M. Kaufman ◽  
Rochelle M. Hines ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Receptor Signaling ◽  
Early Increase

scholarly journals Early Increase in Extrasynaptic NMDA Receptor Signaling and Expression Contributes to Phenotype Onset in Huntington's Disease Mice

Neuron ◽  
2010 ◽  
Vol 65 (3) ◽  
pp. 436 ◽  
Author(s):  
Austen J. Milnerwood ◽  
Clare M. Gladding ◽  
Mahmoud A. Pouladi ◽  
Alexandra M. Kaufman ◽  
Rochelle M. Hines ◽  
...  
Keyword(s):  
Nmda Receptor ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Receptor Signaling ◽  
Early Increase

scholarly journals Proteasome Activator Enhances Survival of Huntington's Disease Neuronal Model Cells

PLoS ONE ◽  
2007 ◽  
Vol 2 (2) ◽  
pp. e238 ◽  
Author(s):  
Hyemyung Seo ◽  
Kai-Christian Sonntag ◽  
Woori Kim ◽  
Elena Cattaneo ◽  
Ole Isacson
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuronal Model ◽  
Proteasome Activator
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