| First Author | Lebrun N | Year | 2021 |
| Journal | Brain Res | Volume | 1772 |
| Pages | 147670 | PubMed ID | 34582789 |
| Mgi Jnum | J:323673 | Mgi Id | MGI:6828494 |
| Doi | 10.1016/j.brainres.2021.147670 | Citation | Lebrun N, et al. (2021) HDAC inhibitor ameliorates behavioral deficits in Mecp2(308/y) mouse model of Rett syndrome. Brain Res 1772:147670 |
| abstractText | Rett syndrome (RTT) is a rare X-linked neurodevelopmental disorder. More than 95% of classic RETT syndrome cases result from pathogenic variants in the methyl-CpG binding protein 2 (MECP2) gene. Nevertheless, it has been established that a spectrum of neuropsychiatric phenotypes is associated with MECP2 variants in both females and males. We previously reported that microtubule growth velocity and vesicle transport directionality are altered in Mecp2-deficient astrocytes from newborn Mecp2-deficient mice compared to that of their wild-type littermates suggesting deficit in microtubule dynamics. In this study, we report that administration of tubastatin A, a selective HDAC6 inhibitor, restored microtubule dynamics in Mecp2-deficient astrocytes. We furthermore report that daily doses of tubastatin A reversed early impaired exploratory behavior in male Mecp2(308/y) mice. These findings are a first step toward the validation of a novel treatment for RTT. |
