Open Access. Powered by Scholars. Published by Universities.®
Articles 1 - 1 of 1
Full-Text Articles in Engineering
Impaired Metabolic Flexibility In A Mouse Model Of Leigh Syndrome, Richard Sterling Mccain Jr
Impaired Metabolic Flexibility In A Mouse Model Of Leigh Syndrome, Richard Sterling Mccain Jr
Theses and Dissertations
Metabolic dysfunction burdens tissues with high energy demands, particularly the brain. Leigh syndrome is a mitochondrial encephalopathy stemming from genetic defects in the electron transport chain. Leigh syndrome patients develop lactic acidosis, ataxia, bilateral necrotizing lesions in the brainstem and basal ganglia, lesion microgliosis, and eventually death due to respiratory failure. The NADH dehydrogenase [ubiquinone] iron-sulfur protein 4 (NDUFS4) knockout mouse is an established model of Leigh syndrome due to impaired assembly of mitochondrial Complex I that develops motoric deficits and necrotizing lesions in the brainstem vestibular nuclei and olfactory bulb. In addition to the Complex I-derived bioenergetics defect, altered …