Early postnatal behavioral, cellular, and molecular changes in models of Huntington disease are reversible by HDAC inhibition.
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ABSTRACT:
SUBMITTER: Siebzehnrubl FA
PROVIDER: S-EPMC6140493 | biostudies-literature | 2018 Sep
REPOSITORIES: biostudies-literature
Publications
Early postnatal behavioral, cellular, and molecular changes in models of Huntington disease are reversible by HDAC inhibition.
Siebzehnrübl Florian A FA Raber Kerstin A KA Urbach Yvonne K YK Schulze-Krebs Anja A Canneva Fabio F Moceri Sandra S Habermeyer Johanna J Achoui Dalila D Gupta Bhavana B Steindler Dennis A DA Stephan Michael M Nguyen Huu Phuc HP Bonin Michael M Riess Olaf O Bauer Andreas A Aigner Ludwig L Couillard-Despres Sebastien S Paucar Martin Arce MA Svenningsson Per P Osmand Alexander A Andreew Alexander A Zabel Claus C Weiss Andreas A Kuhn Rainer R Moussaoui Saliha S Blockx Ines I Van der Linden Annemie A Cheong Rachel Y RY Roybon Laurent L Petersén Åsa Å von Hörsten Stephan S
Proceedings of the National Academy of Sciences of the United States of America 20180827 37
Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by expanded CAG repeats in the <i>huntingtin</i> gene (<i>HTT</i>). Although mutant HTT is expressed during embryonic development and throughout life, clinical HD usually manifests later in adulthood. A number of studies document neurodevelopmental changes associated with mutant <i>HTT</i>, but whether these are reversible under therapy remains unclear. Here, we identify very early behavioral, molecular, and cellu ...[more]
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