Disease models of Leigh syndrome: From yeast to organoids.

Abstract excerpt

Leigh syndrome (LS) is a severe mitochondrial disease that results from mutations in the nuclear or mitochondrial DNA that impairs cellular respiration and ATP production. Mutations in more than 100 genes have been demonstrated to cause LS. The disease most commonly affects brain development and function, resulting in cognitive and motor impairment. The underlying pathogenesis is challenging to ascertain due to...

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• Caenorhabditis elegans
• Drosophila melanogaster
• Electron Transport Complex I
• Induced Pluripotent Stem Cells
• Leigh Disease