Jiou Wang Ph.D
Assistant Professor of Biochemistry and Molecular Biologyjiouw@jhmi.edu
Telephone Number: 410-502-0927
Fax Number: 410-955-2926
Johns Hopkins University
Bloomberg School of Public Health
615 N. Wolfe Street
Baltimore, Maryland 21205
Mechanisms of Neurodegeneration and Protein Quality Control
Neurodegeneration is a poorly understood biomedical phenomenon and a major public health challenge in our increasingly aging society. Our recent work on an inherited form of Amyotrophic Lateral Sclerosis (ALS), which is caused by mutations in SOD1, has shown that protein misfolding may play a central role in the degeneration of motor neurons. It has become clear that further elucidation of the mechanisms of protein misfolding and quality control in neurons is a critical step toward understanding this form of ALS as well as many other neurodegenerative diseases, including Alzheimer’s, Parkinson’s, and Huntington’s. Our goal is to describe at the molecular and cellular levels how specific neurons degenerate, how protein folding and misfolding operate in the cell, and how protective systems fail at disease stages.
To dissect the complex processes of neurodegeneration, we have established novel C. elegans and mouse models of ALS, which recapitulate major features of the disease, providing a unique avenue for our research. Using the C. elegans model and forward genetic screens, we are searching for key genes and pathways of neurodegeneration that are conserved in humans.
New discoveries of disease genes in humans are accelerating our understanding of the molecular underpinnings of neurodegeneration. We use molecular and cellular, genetic, and biochemical approaches to elucidate how these genes function normally and how mutations lead to disease. The elucidation may ultimately lead to better treatments of these devastating diseases.