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  Cortical circuit alterations precede motor impairments in Huntington's disease mice

Burgold, J., Schulz-Trieglaff, E. K., Voelkl, K., Gutierrez-Angel, S., Bader, J. M., Hosp, F., et al. (2019). Cortical circuit alterations precede motor impairments in Huntington's disease mice. Scientific Reports, 9: 6634. doi:10.1038/s41598-019-43024-w.

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Item Permalink: http://hdl.handle.net/21.11116/0000-0003-E220-9 Version Permalink: http://hdl.handle.net/21.11116/0000-0003-E221-8
Genre: Journal Article

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 Creators:
Burgold, Johanna1, Author
Schulz-Trieglaff, Elena Katharina1, Author
Voelkl, Kerstin1, Author
Gutierrez-Angel, Sara1, Author
Bader, Jakob Maximilian2, Author              
Hosp, Fabian2, Author              
Mann, Matthias2, Author              
Arzberger, Thomas1, Author
Klein, Ruediger1, Author
Liebscher, Sabine1, Author
Dudanova, Irina1, Author
Affiliations:
1external, ou_persistent22              
2Mann, Matthias / Proteomics and Signal Transduction, Max Planck Institute of Biochemistry, Max Planck Society, ou_1565159              

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Free keywords: TRANSGENIC MOUSE MODEL; DENDRITIC SPINE; VISUAL-CORTEX; CAG REPEAT; REVEALS; NEURONS; DEFICITS; HD; INTERNEURONS; PREMANIFESTScience & Technology - Other Topics;
 Abstract: Huntington's disease (HD) is a devastating hereditary movement disorder, characterized by degeneration of neurons in the striatum and cortex. Studies in human patients and mouse HD models suggest that disturbances of neuronal function in the neocortex play an important role in disease onset and progression. However, the precise nature and time course of cortical alterations in HD have remained elusive. Here, we use chronic in vivo two-photon calcium imaging to longitudinally monitor the activity of identified single neurons in layer 2/3 of the primary motor cortex in awake, behaving R6/2 transgenic HD mice and wildtype littermates. R6/2 mice show age-dependent changes in cortical network function, with an increase in activity that affects a large fraction of cells and occurs rather abruptly within one week, preceeding the onset of motor defects. Furthermore, quantitative proteomics demonstrate a pronounced downregulation of synaptic proteins in the cortex, and histological analyses in R6/2 mice and human HD autopsy cases reveal a reduction in perisomatic inhibitory synaptic contacts on layer 2/3 pyramidal cells. Taken together, our study provides a time-resolved description of cortical network dysfunction in behaving HD mice and points to disturbed excitation/inhibition balance as an important pathomechanism in HD.

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Language(s): eng - English
 Dates: 2019
 Publication Status: Published online
 Pages: 13
 Publishing info: -
 Table of Contents: -
 Rev. Method: -
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Title: Scientific Reports
  Abbreviation : Sci. Rep.
Source Genre: Journal
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Publ. Info: London, UK : Nature Publishing Group
Pages: - Volume / Issue: 9 Sequence Number: 6634 Start / End Page: - Identifier: ISSN: 2045-2322
CoNE: https://pure.mpg.de/cone/journals/resource/2045-2322