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  Cortical and striatal circuits in Huntington's disease

Blumenstock, S., & Dudanova, I. (2020). Cortical and striatal circuits in Huntington's disease. Frontiers in Neuroscience, 14: 82. doi:10.3389/fnins.2020.00082.

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© 2020 Blumenstock and Dudanova.

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 Creators:
Blumenstock, Sonja1, 2, Author              
Dudanova, Irina2, Author              
Affiliations:
1Department: Molecules-Signaling-Development / Klein, MPI of Neurobiology, Max Planck Society, ou_1113546              
2Research Group: Molecular Neurodegeneration / Dudanova, MPI of Neurobiology, Max Planck Society, ou_3060199              

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Free keywords: R6/2 MOUSE MODEL; AGE-DEPENDENT ALTERATIONS; MEDIUM SPINY NEURONS; MUTANT HUNTINGTIN; BASAL GANGLIA; SYNAPTIC-TRANSMISSION; CANNABINOID RECEPTORS; GLUTAMATE TRANSPORT; DOPAMINE RELEASE; PARVALBUMIN INTERNEURONSHuntington's disease; cortex; basal ganglia; neural circuits; genetic mouse models; in vivo calcium imaging; optogenetics;
 Abstract: Huntington's disease (HD) is a hereditary neurodegenerative disorder that typically manifests in midlife with motor, cognitive, and/or psychiatric symptoms. The disease is caused by a CAG triplet expansion in exon 1 of the huntingtin gene and leads to a severe neurodegeneration in the striatum and cortex. Classical electrophysiological studies in genetic HD mouse models provided important insights into the disbalance of excitatory, inhibitory and neuromodulatory inputs, as well as progressive disconnection between the cortex and striatum. However, the involvement of local cortical and striatal microcircuits still remains largely unexplored. Here we review the progress in understanding HD-related impairments in the cortical and basal ganglia circuits, and outline new opportunities that have opened with the development of modern circuit analysis methods. In particular, in vivo imaging studies in mouse HD models have demonstrated early structural and functional disturbances within the cortical network, and optogenetic manipulations of striatal cell types have started uncovering the causal roles of certain neuronal populations in disease pathogenesis. In addition, the important contribution of astrocytes to HD-related circuit defects has recently been recognized. In parallel, unbiased systems biology studies are providing insights into the possible molecular underpinnings of these functional defects at the level of synaptic signaling and neurotransmitter metabolism. With these approaches, we can now reach a deeper understanding of circuit-based HD mechanisms, which will be crucial for the development of effective and targeted therapeutic strategies.

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Language(s): eng - English
 Dates: 2020-02-06
 Publication Status: Published in print
 Pages: 18
 Publishing info: -
 Table of Contents: -
 Rev. Type: Peer
 Identifiers: ISI: 000515870000001
DOI: 10.3389/fnins.2020.00082
 Degree: -

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Project name : European Research Council (grant FP7 GA ERC-2012-SyG_318987–ToPAG)
Grant ID : 318987
Funding program : Funding Programme 7 (FP7)
Funding organization : European Commission (EC)

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Title: Frontiers in Neuroscience
  Other : Front Neurosci
Source Genre: Journal
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Publ. Info: Lausanne, Switzerland : Frontiers Research Foundation
Pages: - Volume / Issue: 14 Sequence Number: 82 Start / End Page: - Identifier: ISSN: 1662-4548
ISSN: 1662-453X
CoNE: https://pure.mpg.de/cone/journals/resource/1662-4548