Induced pluripotent stem cells derived from patients carrying mitochondrial mutations exhibit altered bioenergetics and aberrant differentiation potential.

Given that mitochondrial function is a highly dynamic process and SRC represents the mitochondrial capacity to meet extra-energy requirements, beyond the basal level; our findings correlating the SRC with clinical presentation and survival in the generated hiPSC lines are highly significant in the development of relevant in vitro models to better understand the origins of LS and understanding mitochondrial dysfunction in early development stages of LS patients.

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