Huntington's disease articles from across Nature Portfolio

Using brain organoids models, Prigione and colleagues uncovered the impact of Huntington’s disease on human brain developmental and identified early dysregulation of CHCHD2, which disrupted mitochondria and might serve as a therapeutic target.

Huntington’s disease (HD) is a neurodegenerative disease that shows selective regional vulnerability. Here, the authors show that postmortem brain HD astrocytes are regionally diverse, with a striatal disease-associated state and a cortical compensatory state that mitigated neural death.

Mosaic organoids where pathological and healthy cells are grown together, reveal the rescue of phenotypes in pathological cells due to communication with healthy cells without harming them, as demonstrated by single-cell RNA-sequencing data.

A study of cognitive reserve in neurodegenerative disease combining drift diffusion models with MRI highlights a reorganization of decision-making processes correlated to left parietal and hippocampal hypertrophy.

By analyzing human samples and multiple mouse models of Huntington’s disease, we found that complement proteins and microglia mediate early and selective loss of corticostriatal synapses. Strategies that block this process can reduce synaptic loss, increase excitatory input to the striatum and prevent the development of cognitive deficits in mice.

Preventing transient defects in postnatal cortical circuit function limits disease progression in a mouse model of Huntington disease.