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03/10/2023 - 15:00

Vall d'Hebron Talks by VHIR 'Molecular Insights into the Pathogenesis of Cerebral Amyloid Angiopathy (CAA): What we can learn from familial Dutch-type CAA'

Ubicació Sala de actos de la planta 10 del Hospital General
Etiqueta Vall d'Hebron Talks by VHIR

Speaker: Dr. William E. Van Nostrand, is the Co-Executive Director of the George & Anne Ryan Institute for Neuroscience, Herrmann Professor of Neuroscience, Professor in the Department of Biomedical and Pharmaceutical Sciences at the University of Rhode Island. He has been working in Alzheimer’s disease and related disorders (ADRD) research for more than 30 years and was the first to purify and characterize amyloid precursor protein (APP), the progenitor of amyloid-beta (Ab).  Dr. Van Nostrand’s laboratory investigates pathogenic mechanisms in ADRD with a focus on cerebral amyloid angiopathy and cerebral small vessel disease, aspects of vascular mediated cognitive impairment and dementia (VCID), which are key elements of ADRD. His laboratory also generates and refines rodent models of these disorders as investigational platforms for pathogenesis, biomarker development, and therapeutic testing. 

Cerebral amyloid angiopathy (CAA), is a prevalent cerebral small vessel disease (CSVD), prominent contributor to vascular cognitive impairment and dementia (VCID) in the elderly, and the most common vascular comorbidity in Alzheimer’s disease (AD). CAA is characterized by deposition of amyloid â (Ab) fibrils in cerebral blood vessels leading to cerebral infarction, cerebral micro- and macro-hemorrhages, white matter damage and cognitive decline. In addition to AD and sporadic forms of this condition, several familial forms of CAA exist resulting from mutations that reside within the Ab peptide region of APP gene. The first familial CAA mutation identified in Ab was the Dutch E22Q variant. Dutch-type CAA patients exhibit early-onset primarily larger vessel CAA type-2 with the noticeable absence of parenchymal amyloid plaques commonly found in AD. Dutch-type CAA patients develop recurrent, and often fatal, intracerebral hemorrhages and cognitive impairment. Modeling familial Dutch-type CAA provides the opportunity to understand how this mutation drives the specific formation of vascular amyloid and the vascular-associated pathology characteristic of CAA. The objectives of this presentation are two-fold: First, to present new structural and functional studies as to how the Dutch E22Q mutation, and other CAA-related mutations, affect the properties of the Ab peptide to promote fibril assembly. Second, to present studies on the generation of a transgenic rat model of Dutch-type CAA that provide new insights into the molecular pathogenesis of this disease. 

Host: Dr. Mar Hernández Guillamón; Main researcher Neurovascular Diseases (VHIR)

Registre Online: https://gencat.zoom.us/webinar/register/WN_xhqiZHOLQlSrmIQhJkFIgA