Ernesto R Bongarzone, PhD
Professor
Mentor, Biological Mechanisms
Department of Anatomy and Cell Biology
Contact
Building & Room:
COMRB 7035
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We identify and characterize pathogenic mechanisms affecting lysosome-autophagy function and transfer basic findings into therapeutic targets applicable for multiple sclerosis, leukodystrophies, Parkinson’s disease, and chronic traumatic encephalopathy.
About
Proteopathies are a collection of adult-onset neurodegenerative conditions where abnormal folding of one or more proteins lead to neuronal and glial dysfunction. Alpha-synuclein (a-syn) is a small (~18 KDa) and soluble pre-synaptic protein of unknown function, and has an intrinsic tendency to form insoluble amyloid aggregates in a number of late onset proteopathies such as Parkinson's disease and multiple system atrophy. Dr. Bongarzone's overarching hypothesis is that a progressive albeit non-lethal deficiency in the homeostatic control of amyloidogenic proteins and sphingolipids by the lysosomal/autophagy pathway is a contributing factor to neuronal vulnerability in aging and adult-onset proteopathic neurodegeneration, including Parkinson's, Alzheimer's, amyotrophic lateral sclerosis.