Topic: Huntingtin

Huntington's disease (HD) is a genetic neurodegenerative disorder, caused by CAG repeat expansion (>39) coding for poly-glutamine (polyQ) tract in the Huntingtin (HTT) gene. Although HD is late onset, it was shown to have an early neurodevelopmental component. In addition, DNA methylation was shown in adults to be an important epigenetic mechanism involved in HD. Here we sought to explore early pre-symptomatic neurodevelopmental alterations in DNA methylation using cerebral organoids from juvenile forms of isogenic HD pluripotent cells. We find wide-spread DNA demethylation in the mutant ce

Age-related neurodegenerative diseases are becoming more prevalent as human lifespan increases. Modeling human aging is challenging in the lab. We use transdifferentiation via an all-in-one lentiviral vector to directly reprogram human donor-derived fibroblasts into induced neurons (iN). The advantage of this methodology is the maintaining of age and disease-related signatures of the donor cells as iNs bypass the intermediary cell rejuvenation.Huntington’s disease (HD) is an autosomal dominant age-related neurodegenerative disorder caused by a mutated Huntingtin (HTT) gene containing an expans