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Upcoming Seminars

Elvira De Leonibus, PhD - "Targeting early disease’ mechanisms in neurodegenerative disorders: the special case of mucopolysaccharidosis type IIIA"

Head of Behavioral Core, TIGEM and PI Neuropsychopharmacology Lab, Institute of Cellular Biology and Neurobiology, National Research Council, Italy
When Apr 02, 2019
from 12:00 PM to 01:30 PM
Where Tigem, Vesuvius Auditorium
Contact Name
Contact Phone 081-19230659
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Abstract
Neurodegenerative diseases are debilitating and largely untreatable conditions. Most of them, such as Parkinson’ and Alzheimer’ disease, are strongly linked with age; inherited neurodegenerative lysosomal storage disorders, such as mucopolysaccharidosis type III (MPS-IIIA), manifest during childhood.
One of the biggest challenges, to fight neurodegenerative disorders, is the identification of early signs of the pathology and associated disease’ mechanisms leading to neuronal loss to develop restorative or protective therapeutic approaches.
I will show how we address this challenge with a novel approach that uses early discrete behavioural dysfunctions to pinpoint synaptic and molecular disease’ mechanisms, while accounting for their intrinsic heterogeneity in the complex and evolving phenotypes of neurodegenerative disorders.
I will show how this approach allowed us to identify completely novel disease’ pathways in Parkinson’ disease and MPS-IIIA and how this approach permits the “repositioning” of treatments among different neurodegenerative and other chronic diseases.

Florian Sennlaub, MD, PhD - "How genetic-risk variants of age–related macular degeneration shape pathogenic inflammation"

Sorbonne Université, INSERM, CNRS, Institut de la Vision, Paris, France
When Apr 09, 2019
from 12:00 PM to 01:30 PM
Where Tigem, Vesuvius Auditorium
Contact Name
Contact Phone 081-19230659
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Abstract
CD47 activation by Thrombospondin 1 (TSP-1) is essential in maintaining the subretinal immunosuppressive environment and prevents the subretinal accumulation of macrophages. Age-related Macular Degeneration (AMD), a highly heritable, major cause of blindness, is characterized by the breakdown of the immune-suppression and an accumulation of pathogenic MPs. Of all genetic factors, a variant of Complement factor H (CFH) and a risk haplotype of 10q26 are associated with greatest linkage to AMD. We recently showed that complement factor H (CFH) and in particular the AMD-associated CFH variant, curbs Thrombospondin 1 (TSP-1) activation of CD47. We now demonstrate that monocytes from homozygous carriers of the major AMD-risk haplotype of the 10q26 locus significantly overexpress the High-Temperature Requirement A Serine Peptidase 1 (HTRA1). Mechanistically we demonstrate that HTRA1 hydrolyses TSP-1, preventing its ability to activate CD47 and induce MP elimination. Our study reveals a comprehensive mechanism how CFH and HTRA1 participate in the pathogenesis of AMD and opens new therapeutic avenues to restore subretinal immunosuppressivity and inhibit the pathogenic subretinal inflammation.

Alejo Efeyan, PhD - "Nutrient Signaling in Health and Disease"

Group Leader, Spanish National Cancer Research Center, CNIO, Madrid, Spain
When May 14, 2019
from 12:00 PM to 01:30 PM
Where Tigem, Vesuvius Auditorium
Contact Name
Contact Phone 081-19230659
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David Ron, MD - "Mechanisms that maintain protein folding homeostasis in the endoplasmic reticulum"

Professor of Cellular Pathophysiology and Clinical Biochemistry, Cambridge Institute for Medical Research, University of Cambridge, England (UK)
When May 28, 2019
from 12:00 PM to 01:30 PM
Where Tigem, Vesuvius Auditorium
Contact Name
Contact Phone 081-19230659
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Short CV

Abstract
A transcriptional and translational program that strives to match the complement of chaperones in the endoplasmic reticulum (ER) to the burden of unfolded proteins in the early secretory pathway (the Unfolded Protein Response or UPR) has long been known to exist and its functional importance has been showcased by genetic and pharmacological manipulations.
However, a homeostatic program centered solely on changes in the gene expression encounters an inherent latency imposed by the lag between changes in the internal milieu of the ER and rectifying changes in levels of UPR target mRNAs and proteins.
Here I will discuss how this problem might be resolved by post-translational adaptations that operate on a shorter time scale