Hibiki Kawamata Fujita, Ph.D.
Assistant Research Professor of Neuroscience
My research interests are in the molecular mechanisms underlying organelle dysfunction in neurodegenerative diseases. My current work focuses on the hypothesis that redox protein folding in the endoplasmic reticulum is dysfunctional in cells harboring mutations associated with amyotrophic lateral sclerosis (ALS). I want to determine how protein misfolding and ER oxidative stress can lead to changes in other ER functions, such as calcium signaling and protein secretion. Another closely related area that I am investigating is the involvement of ER and mitochondrial communications in ALS. Since the two organelles share and depend on physical and functional interactions, in disease conditions this tight contact can be a critical point involved in the pathogenic process. The goals are to determine if the ER-mitochondrial communications are disrupted in various forms of familial and in sporadic ALS. To address these questions I use cell biology, live cell functional microscopy, and molecular biology, in mouse models of ALS as well as in primary cells from mice, primary human fibroblasts, and iPSC-derived motor neurons and astrocytes.