Friday, September 13, 2019 11:30am to 1pm
About this Event
The Harry and Jeanette Weinberg Building at 1741 Ashland Avenue, Baltimore, MD 21205
https://www.eventbrite.com/e/guest-lecture-albert-l-misko-md-phd-tickets-68026863179*This event is for Faculty and Staff*
Dr. Albert L. Misko, MD, PhD will be lecturing on Isolated sulfite oxidase deficiency (ISOD) and molybdenum cofactor deficiency (MoCD) are identical neurodegenerative disorders which closely mimic neonatal hypoxic ischemic encephalopathy (HIE). This similarity suggests a common pathophysiology and raises the possibility that ISOD and MoCD could serve as genetic models of neonatal HIE. To further qualify this potential, we analyzed brain MRIs from a cohort of ISOD and MoCD patients and found that the evolution of radiographic abnormalities is consistent with catastrophic energy failure and excitotoxicity. We further identified a common pathomechanism that converges at NMDA receptor activation and mitochondrial calcium overload. Our results suggest that ISOD and MoCD offer a novel paradigm to study excitotoxicity in the neonatal brain and test neuroprotective agents.