At Paris Brain Institute
Decoding the role of immunity in Charcot’s disease
Séverine Boillée studies how immune cells (or inflammatory cells) influence motor neuron degeneration and disease progression. In ALS, there are more inflammatory cells around the motor neuron than in subjects who do not have the disease. These cells react to the degeneration of motor neurons and are involved in the progression of Charcot’s disease. In the brain and spinal cord, motor neurons interact with microglial cells, while in the peripheral nervous system they are in contact with macrophages. These two types of cells play a dual role: a positive role, by releasing agents beneficial for the survival of motor neurons, and a negative role, via toxic agents that will contribute to their destruction. The work by Séverine Boillée and Christian Lobsiger aims to better understand the role these cells play in the development and progression of Charcot’s disease, to identify new therapeutic approaches. The aim is to specifically analyze the different agents released by these cells and to identify which to act on to slow the progression of the disease.
Early brain impairments in individuals at risk of developing ALS
A study led by Paris Public Hospital Network (AP-HP) and conducted at Paris Brain Institute at Pitié-Salpêtrière Hospital by Isabelle Le Ber within Alexandra Durr and Giovanni Stevanin’s team, and Olivier Colliot co-team leader with Stanley Durrleman has found, for the first time, that asymptomatic individuals at risk of developing frontotemporal degeneration (FTD) or amyotrophic lateral sclerosis (ALS) because they carry the C9ORF72 gene have very early cognitive, anatomical and structural impairments, before the age of 40. Identifying biomarkers at very early stages is a first step towards developing the tools needed to evaluate new treatments. Developing early-stage therapeutics, ideally before the onset of symptoms, requires the development of tools that can tell us when to begin treatments, and to measure their efficacy.
