image: This image illustrates that in rats transgenic (+/+) for Huntington disease (HD) the expression of an important receptor (dopamine D1) is reduced already at postnatal day 10 in this animal model. At this young age, the health status and phenotype of this HD animal model is long before onset of the typical HD-like symptoms become overt at 9-12 month-of-age. view more
Credit: PNAS
Researchers report that rodents with the mutant huntingtin gene exhibited reduced anxiety, as indicated by reduced vocalization and increased risk taking; reduced generation of neurons and oligodendrocytes from progenitor and stem cells in vitro; and imbalances in brain neurotransmitter signaling, compared with controls carrying the wild-type huntingtin gene; presymptomatic treatment with the histone deacetylase inhibitor LBH589 reversed some of the molecular, cellular, and behavioral changes tied to the mutant gene, suggesting a potential early therapeutic approach for Huntington disease, the symptoms of which typically manifest during adulthood.
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Article #18-07962: "Early postnatal behavioral, cellular, and molecular changes in models of Huntington disease are reversible by HDAC inhibition," by Florian A. Siebzehnrubl et al.
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Journal
Proceedings of the National Academy of Sciences