Alexey Pshezhetsky | Pathological Cascades In Neurological Pathological Cascades In Neurological MPS disorders

Le CERMO-FC et la Pre Cathy Vaillancourt ont le plaisir de vous inviter à la présentation du Pr Alexey Pshezhetsky, professeur à l’Université de Montréal. Ses travaux de recherche vous seront présentés par visioconférence le Jeudi 14 octobre 2021 à 12h00.

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Portrait du Pr Alexey Pshezhetsky

CERMO-FC and Pre Cathy Vaillancourt are pleased to invite you to the presentation of Prof. Alexey Pshezhetsky, professor at Université de Montréal. His research work will be presented to you by videoconference on Thursday, October 14, 2021 at 12 noon.

Attend the conference in person?
Due to sanitary measures, places are limited. To reserve your place, please reserve your place here

Portrait of the Pr Alexey Pshezhetsky

Pathological cascades in neuropathic MPS disorders: neuroinflammation, synaptic dysfunction and defects of protein folding and transport. 

Lysosomal storage diseases are hereditary conditions of children caused by inborn errors in lysosomal catabolism. They constitute a heavy burden on the health care system despite their low frequency; the hospitalization and caregiving expenses per patient are upwards of $1 million annually. More than 30% of all lysosomal diseases are mucopolysaccharidoses (MPS), disorders affecting the enzymes required for stepwise degradation of glycosaminoglycans (mucopolysaccharides). Seven MPS are caused by genetic deficiencies of the enzymes involved in heparan sulfate catabolism and lead to neurodegeneration, childhood dementia and death before adulthood.

In my presentation I will summarize recent studies revealing the pathological basis underlying the neurological manifestations in MPS. Together these data provide evidence that accumulation of heparan sulfate triggers multifaceted mechanism leading to neuronal disfunction and death. The mechanism involves general inflammation reactions, release of multifunctional cytokines and oxidative stress, pathological changes in the synapse, mitochondrial system and vesicular transport, progressive accumulation of lipid and protein aggregates and closely resembles pathological cascades in age-related neurodegenerative tauopathies.

Un évènement organisé dans le cadre des Pause-conférences du CERMO-FC
An event organized as part of the Pause-conférences of CERMO-FC