NIH Research Festival
In osteogenesis imperfecta (OI) cell stress in osteoblasts is caused by misfolded procollagen, a result of a Glycine substitution. The impact of this cell stress is seen in the bone pathology generally associated with OI. This study examines mitochondrial dynamics and ROS and ATP production at different points of differentiation between primary osteoblast cultures with and without the G610C mutation, a model OI mouse. This is accomplished through live cell imaging and fluorescent staining. So far, we have seen altered mitochondrial fission, increased ROS production in differentiating osteoblasts, and reduced ATP synthesis in mature cells. This suggests that ISR is activated due to the blockage of ER exit sites and the subsequent disruption of Er-Mitochondrial contact.
Scientific Focus Area: Cell Biology
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