Mitochondrial DNA density homeostasis accounts for a threshold effect in a cybrid model of a human mitochondrial disease.

ABSTRACT: Mitochondrial dysfunction is involved in a wide array of devastating diseases, but the heterogeneity and complexity of the symptoms of these diseases challenges theoretical understanding of their causation. With the explosion of omics data, we have the unprecedented opportunity to gain deep understanding of the biochemical mechanisms of mitochondrial dysfunction. This goal raises the outstanding need to make these complex datasets interpretable. Quantitative modelling allows us to translate such datasets into intuition and suggest rational biomedical treatments. Taking an interdisciplinary approach, we use a recently published large-scale dataset and develop a descriptive and predictive mathematical model of progressive increase in mutant load of the MELAS 3243A>G mtDNA mutation. The exper

SUBMITTER: Aryaman J 

PROVIDER: S-EPMC5705840 | biostudies-literature | 2017 Nov

REPOSITORIES: biostudies-literature