Drug significantly reduces chorea symptoms in patients with Huntington’s disease

The drug valbenazine statistically improves chorea, a movement disorder commonly associated with Huntington’s disease, when compared to a placebo, according to a recent international study led by UTHealth Houston researcher Erin Furr Stimming, MD, who served as principal investigator on behalf of the KINECT-HD Huntington Study Group.

UC Irvine-led study links metabolism changes in certain brain cells to Huntington’s disease

Irvine, Calif., Dec. 21, 2022 — A research team led by the University of California, Irvine has linked the mutation that causes Huntington’s disease to developmental deficits in the brain’s oligodendrocyte cells that are caused by changes in metabolism. They found that high doses of thiamine and biotin can restore normal processes.

UCI researchers discover crucial role of brain’s striatum cilia in time perception

Irvine, Calif., Nov. 30, 2022 — Researchers at the University of California, Irvine have discovered that removal of cilia from the brain’s striatum region impaired time perception and judgment, revealing possible new therapeutic targets for mental and neurological conditions including schizophrenia, Parkinson’s and Huntington’s diseases, autism spectrum disorder, and Tourette syndrome.

Cellular housekeeping process implicated in fatal neurological disorder

A new study from Washington University School of Medicine in St. Louis indicates that as patients age, Huntington’s disease gradually impairs autophagy, which eliminates waste from cells. This housekeeping is significant because a buildup of waste in a specific kind of neuron leads to such cells’ untimely deaths. The researchers also showed that enhancing the autophagy pathway in such neurons that were created from skin cells of Huntington’s patients protects those cells from dying.

Brain Cell Activity Plays Critical Role in CNS Disorder Outcomes

Investigators at Cedars-Sinai have comprehensively mapped molecular activity in the brain and spinal cord that is responsible for regulating the body’s response to central nervous system (CNS) disorders such as Alzheimer’s, Huntington’s disease and spinal cord injuries.

Hereditary Disease Foundation Announces 2021 Prizes Recognizing Leadership in Huntington’s Disease Research

The Hereditary Disease Foundation today announced the recipients of its 2021 Awards: Elena Cattaneo, Director of the Laboratory of Stem Cell Biology and Pharmacology of Neurodegenerative Diseases at the University of Milan, will receive the Hereditary Disease Foundation Leslie Gehry Brenner Prize for Innovation in Science. Sarah Hernandez, postdoctoral fellow and project scientist in the Thompson Lab at University of California, Irvine, will receive the Nancy S. Wexler Young Investigator Prize.

Gene Editing Therapy in Early Stages of Huntington’s Disease May Slow Down Symptom Progression, Mouse Study Shows

In a new study on mice, Johns Hopkins Medicine researchers report that using MRI scans to measure blood volume in the brain can serve as a noninvasive way to potentially track the progress of gene editing therapies for early-stage Huntington’s disease, a neurodegenerative disorder that attacks brain cells. The researchers say that by identifying and treating the mutation known to cause Huntington’s disease with this type of gene therapy, before a patient starts showing symptoms, it may slow progression of the disease.

New study finds abnormal response to cellular stress is associated with Huntington’s disease

A new University of California, Irvine-led study finds that the persistence of a marker of chronic cellular stress, previously associated with neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), also takes place in the brains of Huntington’s disease (HD) patients.

Protein Linked to ALS/Ataxia Could Play Key Role in Other Neurodegenerative Disorders

A new study suggests that some neurological disorders share a common underlying thread. Staufen1, a protein that accumulates in the brains of patients with certain neurological conditions, is linked to amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease, along with other neurological disorders, including Alzheimer’s, Parkinson’s, and Huntington’s disease, according to University of Utah Health scientists.

What’s It All About? Alfy and Aggregates: A Free Webinar from the Hereditary Disease Foundation

The Hereditary Disease Foundation is hosting a free webinar “What’s It All About? Alfy and Aggregates” on Tuesday, March 23, 2021, from 12pm to 1pm ET. Dr. Ai Yamamoto, associate professor in the departments of Neurology and of Pathology and Cell Biology at Columbia University Vagelos College of Physicians and Surgeons and recipient of the 2020 Leslie Gehry Brenner Prize for Innovation in Science, will be the keynote speaker.