News Release

New insights into Huntington disease – Looking at a neurodegenerative disorder from a neurodevelopmental perspective

The disorder long thought to manifest its neurodegenerative symptoms in older age, now appears to have effects beginning in-utero

Meeting Announcement

Federation of European Neuroscience Societies

Paris, France – July 8, 2022: Dr. Sandrine Humbert from the Grenoble Institute of Neurosciences will present revolutionary work done on Huntington’s disease from 5:30-6:30 pm on July 10, 2022, at the FENS 2022 Forum.

Huntington disease is a rare genetic disorder affecting 5-10 people per 100,000 people in Europe. The degenerative nature of the disorder causes psychiatric, cognitive, and motor symptoms that include depression, anxiety, and obsessive-compulsive tendencies, decline in memory and ability to plan and organise, as well as uncontrolled movement of certain body parts.

All this is caused by a single mutation on the fourth chromosome of our genome. This detrimental mutation causes a mutation in the protein Huntingtin. Though the normal role of this protein is not fully understood, it is thought to play an important role in nerve cells and the effects of its malfunction contribute to Huntington’s disease.

With the effects of Huntington disease being so devastating on patients and their loved ones, researchers around the world work tirelessly to better understand the disorder in the hopes of finding a curative treatment that does not exist currently.

While the symptoms of Huntington disease normally begin in adulthood (after age 30), an important part of better understanding Huntington’s involves looking at exactly what effects the malfunctioning protein may have and just how early malfunctions start occurring.

To look at this, Dr. Humbert and a team of researchers at various French institutions carried out a two-part study in animals and humans. In mice, they were able to find that this malfunctioning protein, impairs how brain cells are formed via neural progenitor cell division and how they migrate to the right place and mature within the central nervous system.

To investigate this further, they also examined tissue from human fetuses that carried the Huntington disease mutation. At only 13 weeks of gestation, these tissues also showed clear abnormalities, specifically in the developing outer layer of the brain (cortex), which we know plays a vital role in the higher-level processes of the human brain such as perception of senses, our emotions, decision-making, language ability, and more.

These disruptive observations confirm that neurodegenerative disorders can have a neurodevelopmental component and they highlight the need for new molecular treatment that could be given to patients very early on in life.

The FENS Forum features a high-quality scientific programme covering all aspects of neuroscience, from basic to translational research. Over the course of five days, attendees will have unprecedented access to a range of symposia, technical workshops, plenary and special lectures like these as well as poster sessions and more!

FENS and the Société des Neurosciences are eager to welcome the neuroscience community to attend Europe's largest international neuroscience meeting on 9-13 July 2022 in Paris, France.

Press passes are still available.

About FENS

FENS is the main organisation for neuroscience in Europe. FENS currently represents 44 European national and single discipline neuroscience societies across 33 European countries and over 21,000 member scientists. FENS promotes neuroscience research to policy-makers, funding bodies and the general public, both regionally and internationally. FENS promotes excellence in neuroscience research and facilitates exchanges and networking between neuroscientists within the European Research Area and beyond.

Media contact before and during the Forum

FENS Forum Press Officer

Michelle Wilson-André

Mw.scicom@gmail.com

+34 7 68 86 55 47

 


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