Junhuang Zou, Ph.D.
Meet the Researcher
Junhuang Zou, Ph.D. received his Ph.D. in Genetics at the Institute of Genetics and Developmental Biology, Chinese Academy of Sciences.
Zou is now a postdoctoral research associate in the John A. Moran Eye Center, University of Utah.
Dr. Zou is the recipient of the C.H.E.A.R. endowment, created to support an annual Sensory-Neural Deafness Research Grant. C.H.E.A.R. (Children Hearing Education and Research) was absorbed into Hearing Health Foundation in 1991, and we are very proud to continue their legacy of funding research in sensory-neural deafness.
The Research
University of Utah
Understanding the function of PDZD7 in hair cells
Usher syndrome is a devastating genetic disease affecting both hearing and vision. It is the leading genetic cause of combined deafness and blindness in the world. Usher syndrome is clinically and genetically heterozygous. It is classified into three clinical types according to the severity of hearing and vestibular symptoms. Zou's studies focus on type 2, which is the dominant form of Usher syndrome and characterized by congenital moderate degree of hearing loss, normal vestibular function, and retinitis pigmentosa. PDZD7 was recently reported to exacerbate the symptoms and contribute the digenic form of Usher syndrome type 2 when patients carry heterozygous PDZD7 mutations. Zou has successfully generated a Pdzd7 knockout mouse. Using this animal model, Zou plans to investigate the function of PDZD7 during hair bundle development in the cochlea. This study will be significant for the future diagnosis and treatments for Usher syndrome type 2.
Research area: Usher Syndrome
Long-term goal of research: To develop mechanism-based treatments for Usher syndrome by understanding the formation of USH protein interactomes and their functions in the development and maintenance of hair bundles in hair cells.