Nav: Home

Reducing mutant Huntington disease protein can restore cognitive function in mice

October 03, 2018

New research from the University of British Columbia suggests that reducing mutated Huntington disease protein in the brain can restore cognitive and psychiatric impairments in mice.

Huntington disease (HD) is a genetic, progressive disorder that causes mental decline, psychiatric problems and uncontrolled movements. The disease is caused by mutant huntingtin (HTT) protein, with symptoms appearing in adulthood and worsening over time.

Scientists delivered gene therapy treatment to mice in the form of pieces of DNA, called antisense oligonucleotides, that decreased production of the toxic HTT protein. With this intervention, the researchers found mice regained cognitive performance and became less anxious and depressed.

"Being able to restore cognitive function and improve anxiety in an ill mouse is very exciting," said study lead author Amber Southwell, assistant professor at Burnett School of Biomedical Sciences, who conducted the research as a postdoctoral research fellow at the UBC Centre for Molecular Medicine and Therapeutics.

"Most Huntington disease studies have focused on motor performance, so we wanted to assess mood, learning and memory that represent significant burdens."

There is no cure for Huntington disease, but researchers are working towards preventative treatment, so people who have the HTT gene never develop the disease. Scientists hope people may one day be able to access ongoing treatment, much like those who suffer from asthma.

Michael Hayden, study senior author, University Killam Professor, senior scientist and founder of the UBC Centre for Molecular Medicine and Therapeutics, said the symptom that is most apparent in HD patients is the uncontrolled dance like movements, but the psychiatric symptoms and cognitive decline are particularly debilitating.

"This study reinforces the potential of huntingtin lowering not only on the movement disorder but also on psychiatric and cognitive features, assuming adequate lowering of huntingtin in the appropriate regions is achieved," said Hayden.

Approximately one in 7,000 people in Canada has HD and every child of a person with the disease has a 50 per cent chance of inheriting the gene that causes the illness.
-end-
The study was published in the journal Science Translational Medicine.

University of British Columbia

Related Protein Articles:

A protein that controls inflammation
A study by the research team of Prof. Geert van Loo (VIB-UGent Center for Inflammation Research) has unraveled a critical molecular mechanism behind autoimmune and inflammatory diseases such as rheumatoid arthritis, Crohn's disease, and psoriasis.
Resurrecting ancient protein partners reveals origin of protein regulation
After reconstructing the ancient forms of two cellular proteins, scientists discovered the earliest known instance of a complex form of protein regulation.
Sensing protein wellbeing
The folding state of the proteins in live cells often reflect the cell's general health.
Protein injections in medicine
One day, medical compounds could be introduced into cells with the help of bacterial toxins.
Discovery of an unusual protein
Scientists from Bremen discover an unusual protein playing a significant role in the Earth's nitrogen cycle.
Protein aggregation: Protein assemblies relevant not only for neurodegenerative disease
Amyloid fibrils play a crucial role in neurodegenerative illnesses. Scientists from Heinrich Heine University Düsseldorf (HHU) and Forschungszentrum Jülich have now been able to use cryo-electron microscopy (cryo-EM) to decode the spatial structure of the fibrils that are formed from PI3K SH3 domains - an important model system for research.
Old protein, new tricks: UMD connects a protein to antibody immunity for the first time
How can a protein be a major contributor in the development of birth defects, and also hold the potential to provide symptom relief from autoimmune diseases like lupus?
Infection-fighting protein also senses protein misfolding in non-infected cells
Researchers at the University of Toronto have uncovered an immune mechanism by which host cells combat bacterial infection, and at the same time found that a protein crucial to that process can sense and respond to misfolded proteins in all mammalian cells.
Quorn protein builds muscle better than milk protein
A study from the University of Exeter has found that mycoprotein, the protein-rich food source that is unique to Quorn products, stimulates post-exercise muscle building to a greater extent than milk protein.
More than a protein factory
Researchers from the Stowers Institute for Medical Research have discovered a new function of ribosomes in human cells that may show the protein-making particle's role in destroying healthy mRNAs, the messages that decode DNA into protein.
More Protein News and Protein Current Events

Trending Science News

Current Coronavirus (COVID-19) News

Top Science Podcasts

We have hand picked the top science podcasts of 2020.
Now Playing: TED Radio Hour

Teaching For Better Humans 2.0
More than test scores or good grades–what do kids need for the future? This hour, TED speakers explore how to help children grow into better humans, both during and after this time of crisis. Guests include educators Richard Culatta and Liz Kleinrock, psychologist Thomas Curran, and writer Jacqueline Woodson.
Now Playing: Science for the People

#556 The Power of Friendship
It's 2020 and times are tough. Maybe some of us are learning about social distancing the hard way. Maybe we just are all a little anxious. No matter what, we could probably use a friend. But what is a friend, exactly? And why do we need them so much? This week host Bethany Brookshire speaks with Lydia Denworth, author of the new book "Friendship: The Evolution, Biology, and Extraordinary Power of Life's Fundamental Bond". This episode is hosted by Bethany Brookshire, science writer from Science News.
Now Playing: Radiolab

Space
One of the most consistent questions we get at the show is from parents who want to know which episodes are kid-friendly and which aren't. So today, we're releasing a separate feed, Radiolab for Kids. To kick it off, we're rerunning an all-time favorite episode: Space. In the 60's, space exploration was an American obsession. This hour, we chart the path from romance to increasing cynicism. We begin with Ann Druyan, widow of Carl Sagan, with a story about the Voyager expedition, true love, and a golden record that travels through space. And astrophysicist Neil de Grasse Tyson explains the Coepernican Principle, and just how insignificant we are. Support Radiolab today at Radiolab.org/donate.