scholarly journals Motor deficits associated with Huntington's disease occur in the absence of striatal degeneration in BACHD transgenic mice

2016 ◽  
Vol 25 (9) ◽  
pp. 1780-1791 ◽  
Author(s):  
Susanna Mantovani ◽  
Richard Gordon ◽  
Rui Li ◽  
Daniel C. Christie ◽  
Vinod Kumar ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Motor Deficits ◽  
Striatal Degeneration

scholarly journals The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice

2008 ◽  
Vol 105 (40) ◽  
pp. 15564-15569 ◽  
Author(s):  
Elizabeth A. Thomas ◽  
Giovanni Coppola ◽  
Paula A. Desplats ◽  
Bin Tang ◽  
Elisabetta Soragni ◽  
...  
Keyword(s):  
Gene Expression ◽  
Transgenic Mice ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Hdac Inhibitor ◽  
Brain Size ◽  
Hdac Inhibitors ◽  
Motor Deficits ◽  
Therapeutic Effects ◽  
Mutant Huntingtin

Transcriptional dysregulation has emerged as a core pathologic feature of Huntington's disease (HD), one of several triplet-repeat disorders characterized by movement deficits and cognitive dysfunction. Although the mechanisms contributing to the gene expression deficits remain unknown, therapeutic strategies have aimed to improve transcriptional output via modulation of chromatin structure. Recent studies have demonstrated therapeutic effects of commercially available histone deacetylase (HDAC) inhibitors in several HD models; however, the therapeutic value of these compounds is limited by their toxic effects. Here, beneficial effects of a novel pimelic diphenylamide HDAC inhibitor, HDACi 4b, in an HD mouse model are reported. Chronic oral administration of HDACi 4b, beginning after the onset of motor deficits, significantly improved motor performance, overall appearance, and body weight of symptomatic R6/2300Q transgenic mice. These effects were associated with significant attenuation of gross brain-size decline and striatal atrophy. Microarray studies revealed that HDACi 4b treatment ameliorated, in part, alterations in gene expression caused by the presence of mutant huntingtin protein in the striatum, cortex, and cerebellum of R6/2300Q transgenic mice. For selected genes, HDACi 4b treatment reversed histone H3 hypoacetylation observed in the presence of mutant huntingtin, in association with correction of mRNA expression levels. These findings suggest that HDACi 4b, and possibly related HDAC inhibitors, may offer clinical benefit for HD patients and provide a novel set of potential biomarkers for clinical assessment.

scholarly journals Conditioned medium from amniotic cells protects striatal degeneration and ameliorates motor deficits in the R6/2 mouse model of Huntington's disease

2018 ◽  
Vol 23 (2) ◽  
pp. 1581-1592 ◽  
Author(s):  
Carmela Giampà ◽  
Alessandra Alvino ◽  
Marta Magatti ◽  
Antonietta R. Silini ◽  
Antonella Cardinale ◽  
...  
Keyword(s):  
Mouse Model ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Conditioned Medium ◽  
Motor Deficits ◽  
Striatal Degeneration ◽  
Amniotic Cells

scholarly journals Calpain Is a Major Cell Death Effector in Selective Striatal Degeneration InducedIn Vivoby 3-Nitropropionate: Implications for Huntington's Disease

2003 ◽  
Vol 23 (12) ◽  
pp. 5020-5030 ◽  
Author(s):  
Nicolas Bizat ◽  
Jean-Michel Hermel ◽  
Frédéric Boyer ◽  
Carine Jacquard ◽  
Christophe Créminon ◽  
...  
Keyword(s):  
Cell Death ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Striatal Degeneration ◽  
Death Effector
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