Jiou Wang, MD
- Associate Professor
- Biochemistry and Molecular Biology (Primary)
615 N. Wolfe Street
Baltimore, Maryland 21205
PhD, Johns Hopkins School of Medicine, 2002
MD, Peking University Health Sciences Center, 1997
Protein and RNA Homeostasis in Neurodegeneration
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, such as TDP-43 and C9orf72, 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.
Postdoctoral positions are available. Please send a CV to firstname.lastname@example.org.
- Ugolino J, Ji YJ, Conchina K, Chu J, Nirujogi RS, Pandey A, Wang Jl. (2016) Loss of C9orf72 Enhances Autophagic Activity via Deregulated mTOR and TFEB Signaling. PLoS Genet 12(11): e1006443.
- Periz G, Lu J, Kankle M, Jablonski A, Kalb R, McCampbell A, Wang J. (2015) Regulation of protein quality control by UBE4B and LSD1 through p53-mediated transcription. PLoS Biology 13(4):e1002114.
- Zhang T, Baldie G, Periz G, Wang J (2014) RNA-Processing Protein TDP-43 Regulates FOXO-Dependent Protein Quality Control in Stress Response. PLoS Genet 10(10): e1004693.
- Haeusler AR, Donnelly C, Periz G, Simko E, Shaw P, Kim M, Maragakis N, Troncoso J, Pandey A, Sattler R, Rothstein J, and Wang J. (2014) C9orf72 Nucleotide Repeat Structures Initiate Molecular Cascade of Disease. Nature 507: 195-200.
- Zhang T, Hwang H.Y., Hao H., Talbot C., Jr., and Wang J. (2012) Caenorhabditis elegans RNA-processing Protein TDP-1 Regulates Protein Homeostasis and Lifespan. J Biol Chem. 287, 8371-8382 (Paper of the Week).