Professor Riccardo Brambilla

Professor Riccardo Brambilla

Pilot Project: Cardiff University, 2024

Comprehensive Proteomic and Phosphoproteomic Profiling in Human and Murine Models of Huntington’s Disease; Investigating the Effects of a Novel ERK-Modulating Cognitive Enhancer. 

A new pathway to treat Huntington’s disease dementia.

Summary  

Professor Riccardo Brambilla and his research team aim to test their newly developed Huntington’s disease treatment on mice and human cells. 

What do we already know?  

Huntington’s disease is a brain disorder which has a wide range of symptoms and gets worse over time. It usually effects people 30-50 years old and it's a genetic disease, which means it runs in families. Generally, Huntington’s disease will affect a person's movement, behaviour and/or cognition. As the disease progresses, Huntington’s disease further damages the brain and can lead to a rare type of dementia called Huntington’s disease dementia.  

Right now, there is no cure for Huntington’s disease dementia so finding a treatment is vital. 

Professor Brambilla’s previous research has found a new pathway that could be targeted to develop treatments for the cognitive decline seen in Huntington’s disease dementia.  

One of the key pathways in the brain, known as the ERK signalling pathway, plays an important role in protecting neurons (nerve cells in the brain) from dying which is a common issue in dementia. This pathway is also essential for learning, memory, and overall brain function. 

Professor Riccardo Brambilla and his team have recently developed a drug that can help to protect brain cells and improve memory in animal models by activating the ERK pathway. 

What is this project trying to find out?  

In this project, Professor Riccardo Brambilla and his team will use this drug to explore how it affects the brain in models of Huntington's disease. They will test the drug on a mouse model of Huntington’s disease which is often used for research. They will also test the drug on neurons which have been made from human stem cells that have been genetically altered to carry the Huntington’s disease mutation. 

By studying these models, the researchers aim to reverse the symptoms related to Huntington’s disease dementia. They also aim to identify specific molecular markers in the brain that may cause the beneficial effects of their drug.  

Why is this important?

By the end of this research, the research team hope to have a better understanding of new drug targets to treat Huntington's disease. 

They believe that their findings will not only improve the development of treatments for Huntington’s Disease dementia but also benefit other dementias, including Alzheimer’s Disease. 

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