The Blood-Brain Barrier Is Unaffected in the Ndufs4^-/- Mouse Model of Leigh Syndrome.

ABSTRACT: Mitochondrial dysfunction plays a major role in physiological aging and in many pathological conditions. Yet, no study has explored the consequence of primary mitochondrial deficiency on the blood-brain barrier (BBB) structure and function. Addressing this question has major implications for pharmacological and genetic strategies aimed at ameliorating the neurological symptoms that are often predominant in patients suffering from these conditions. In this study, we examined the permeability of the BBB in the Ndufs4^-/- mouse model of Leigh syndrome (LS). Our results indicated that the structural and functional integrity of the BBB was preserved in this severe model of mitochondrial disease. Our findings suggests that pharmacological or gene therapy strategies targeting the

SUBMITTER: Reynaud-Dulaurier R 

PROVIDER: S-EPMC11084937 | biostudies-literature | 2024 Apr

REPOSITORIES: biostudies-literature