Researchers found that the mutant huntingtin protein aggregates characteristic of Huntington’s disease have distinctive structural features, a discovery that might help to uncover the disease’s underlying mechanisms and lead to new therapies. The study, “Huntingtin exon 1 fibrils feature an interdigitated β-hairpin–based polyglutamine core,” was published in Proceedings of the National…
A research team successfully developed a virus variant that is more efficient than the current standard at crossing the blood-brain barrier and delivering genes to the central nervous system (CNS), making it a promising gene therapy approach to treating neurodegenerative diseases such as Huntington’s disease. The blood-brain barrier, made up of specialized…
A review published in the journal Nature and Science of Sleep explores the links between sleep disturbance and neurodegeneration in diseases such as Huntington’s disease (HD). The authors state that sleep difficulties might not only be a symptom of the underlying neurodegeneration but also a contributing factor driving…
Texas Tech University Health Sciences Center researchers have identified a molecule that might have protective effects against neuronal damage caused by mutant huntingtin, and constitute a potential therapeutic target for the treatment of Huntington’s disease. The study, “Mitochondrial division inhibitor 1 protects against mutant huntingtin-induced abnormal mitochondrial dynamics, synaptic dysfunction…
The Harrington Discovery Institute at University Hospitals (UH) in Cleveland, Ohio, recently announced the 2016 recipients of its Harrington Scholar-Innovator Awards, given in support of physician-scientists whose discovery research holds promise in advancing the standard of patient care. Among this year’s winners is Stanley N. Cohen, MD, from Stanford University, who received $700,000…
Texas Tech University Health Sciences Center (TTUHSC) researchers have identified a molecule — mitochondrial division inhibitor 1 (Mdivi1) — believed to hold promise as a potential treatment for Huntington’s disease. Mitochondria are specialized organelles in a cell responsible for creating the energy needed by the body to sustain life. An…
Neuropsychiatric symptoms of depression and irritability or aggression are associated with alterations in the white matter structure of the brains of early-onset Huntington’s disease (HD) patients. These are the results of a recent study titled “Neuropsychiatry and White Matter Microstructure in Huntington’s Disease,” published in the…
A study with a small cohort of Huntington’s disease patients differed from previous research in finding no metabolic alterations associated with disease progression in stage 2/3 patients compared to both premanifest and healthy subjects. The study, titled “A Metabolic Study of Huntington’s Disease,” was published in the PLOS One journal.
Max Planck Institute of Biochemistry researchers have shown that the intracellular location of protein aggregates, long suspected to be involved in the pathogenesis of neurodegenerative diseases like Huntington’s, is extremely important to the survival of nerve cells. While location in the nucleus has little influence on cellular function, protein deposits in the…
Scientists have identified a proteomic pathway, involving the proteins Homer-3 and mTORC1, that is involved in the degeneration of Purkinje cells, a pathogenesis central to Huntington’s disease. The research paper, “Impaired mTORC1-Dependent Expression of Homer-3 Influences SCA1 Pathophysiology,” was published in Neuron. Purkinje cells (PCs) are the principal neurons…
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