Translational Neuroscience Research and Reviews

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Table of Content: Volume 3, Issue 1

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Research Article Pages 62-80

Loss of Oligodendrocytes by Oxidative Phosphorylation Inhibitor Rotenone and its Reversal by Phenylbutyrate (PB) in Human Brain Developmental Organoid Model

Authors: David Freedman, PhD, Ewa K Stachowiak, PhD and Michal K Stachowiak, PhD

Abstract: Brain oligodendrocytes have been shown to be particularly affected by hypoxia and mitochondrial insufficiency. To model their effects on human brain development, we exposed developing human iPSC derived cerebral organoids to rotenone, a mitochondrial oxidative phosphorylation (OXPHOS) inhibitor.

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Research Article Pages 53-61

Loss of Oligodendrocytes in Mouse Model of Pyruvate Dehydrogenase Complex Deficiency and Partial Reversal by Phenylbutyrate Treatment

Authors: David Freedman, Ilona Klejbor, Saleh Mahmood, Mulchand S Patel, Michal K Stachowiak and Ewa K Stachowiak

Abstract: Human pyruvate dehydrogenase complex (PDC) deficiency caused by mutations of the pyruvate dehydrogenase component (PDH) of the pyruvate dehydrogenase complex (PDC) is associated with the loss of myelin in the cortex and subcortical brain regions. Similar changes occur in the heterozygous PDH^+/- mouse model of a partial PDC deficiency.

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