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Jiou Wang

Departmental Affiliations

School of Medicine

Jiou Wang, PhD, MD, seeks to understand what controls the birth and death of a protein and how the shape and function of DNA and RNA are connected in health and diseases.

Contact Info

615 N. Wolfe Street, Room E8628

Research Interests

Protein; RNA; neurodegeneration
Experiences & Accomplishments
Johns Hopkins School of Medicine
Peking University Health Sciences Center
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 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. In addition to protein quality control, RNA metabolism is another theme implicate in neurodegenerative diseases. It has become clear that further elucidation of the mechanisms of protein quality control and RNA homeostasis will be critical for understanding not just ALS but also other neurodegenerative diseases. Our goal is to describe at the molecular and cellular levels how protein quality control and RNA homeostasis operate in the cell, why protective systems fail at disease stages, and what could constitute effective interventions.
New discoveries of disease genes, such as C9orf72, TDP-43, and FUS, 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.
Positions for graduate students/postdoctoral fellows/visiting scholars are available. Please send a CV to
Honors & Awards
Elected to The American Society for Clinical Investigation (ASCI) (2020)
Select Publications
Recent publications
  • Wang T, Liu H, Itoh K, Zhang L, Sesaki H, Hartung T, Wang J. (2021) C9orf72 Regulates Energy Homeostasis by Stabilizing Mitochondrial Complex I Assembly. Cell Metabolism doi: 10.1016/j.cmet.2021.01.005
  • Lu J, Periz G, Lu YN, Tang Q, Liu Y, Zhang T, Shah Y, Thombre R, Aljumaah R, Li W, Mojsilovic-Petrovic J, Ji Y, Johnson K, Kalb R, Wang J. (2019) L3MBTL1 regulates ALS/FTD-associated proteotoxicity and quality control. Nature Neuroscience. doi: 10.1038/s41593-019-0384-5.
  • Alexander E, Niaki A, Zhang T, Sarkar J, Liu Y, Nirujogi R, Pandey A, Myong S, Wang J. (2018) Ubiquilin 2 Modulates ALS/FTD-linked FUS-RNA Complex Dynamics and Stress Granule Formation. Proc. Natl. Acad. Sci. USA
  • Zhang T, Wu Y, Mullane P, Ji Y, Liu H, He L, Arora A, Hwang H, Alessi A, Niaki A,Periz G, Guo L, Wang H, Elkayam E, Joshua-Tor L, Myong S, Kim J, Shorter J, Ong S, Leung A, Wang J. (2018) FUS Regulates Activity of MicroRNA-mediated Gene Silencing. Molecular Cell 69:787-801.
  • 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.