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His research interests center on developing novel genetic mouse models of severe mitochondrial dysfunction in retinal ganglion cells (RGCs) and other retinal neurons in order to recapitulate the RGC degeneration seen in human optic neuropathies and the poorly understood pigmentary retinopathy that may accompany these diseases.
Mitochondria are the powerhouse of our cells, efficiently generating energy through oxidative metabolism. When mitochondria function improperly, cells are deprived of needed energy and are subjected to the adverse effects of reactive oxygen species. Mitochondrial dysfunction is an important cause of vision loss and is believed to play a mechanistic role in a number of optic neuropathies, most notably in primary mitochondrial optic neuropathies like Leber hereditary optic neuropathy and dominant optic atrophy, but also secondarily in more common diseases like optic neuritis, ischemic optic neuropathy, and glaucoma. Currently there are no pharmacotherapies for mitochondrial optic neuropathies that are of more than marginal clinical benefit to affected patients.
Dr. Gospe employs biochemical, histologic, and electrophysiological approaches to characterize the metabolic perturbations and aberrant signaling pathways leading to degeneration of retinal neurons in the face of reduced oxidative metabolism. The mutant mouse lines he is developing may serve as useful preclinical models to identify and validate therapeutic targets for future human trials. Ultimately, the hope is that strategies to modulate mitochondrial physiology may be neuroprotective not only in primary mitochondrial optic neuropathies but also in other optic neuropathies causing significant visual morbidity in patients.
Mitochondria are the powerhouse of our cells, efficiently generating energy through oxidative metabolism. When mitochondria function improperly, cells are deprived of needed energy and are subjected to the adverse effects of reactive oxygen species. Mitochondrial dysfunction is an important cause of vision loss and is believed to play a mechanistic role in a number of optic neuropathies, most notably in primary mitochondrial optic neuropathies like Leber hereditary optic neuropathy and dominant optic atrophy, but also secondarily in more common diseases like optic neuritis, ischemic optic neuropathy, and glaucoma. Currently there are no pharmacotherapies for mitochondrial optic neuropathies that are of more than marginal clinical benefit to affected patients.
Dr. Gospe employs biochemical, histologic, and electrophysiological approaches to characterize the metabolic perturbations and aberrant signaling pathways leading to degeneration of retinal neurons in the face of reduced oxidative metabolism. The mutant mouse lines he is developing may serve as useful preclinical models to identify and validate therapeutic targets for future human trials. Ultimately, the hope is that strategies to modulate mitochondrial physiology may be neuroprotective not only in primary mitochondrial optic neuropathies but also in other optic neuropathies causing significant visual morbidity in patients.
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Liam S C Lewis,Nikolai P Skiba,Ying Hao,Howard M Bomze,Vadim Y Arshavsky, Romain Cartoni,Sidney M Gospe
bioRxiv : the preprint server for biology (2024)
Alexander M. Warwick,Howard M. Bomze,Luyu Wang,Mikael Klingeborn,Ying Hao,Sandra S. Stinnett,Sidney M. Gospe
biorxiv(2022)
Journal of Neuro-Ophthalmology (2021)
Journal of American Association for Pediatric Ophthalmology and Strabismusno. 4 (2021): e8-e9
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