The Blood–Brain Barrier Is Unaffected in the INdufs4−/−/I Mouse Model of Leigh Syndrome

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 pharmac...

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Bibliographic Details
Published inInternational journal of molecular sciences Vol. 25; no. 9
Main Authors Reynaud-Dulaurier, Robin, Clément, Romain, Yjjou, Sara, Cresson, Cassandra, Saoudi, Yasmina, Faideau, Mathilde, Decressac, Michael
Format Journal Article
LanguageEnglish
Published MDPI AG 01.05.2024
Subjects
Analysis
Genes
Medical research
Medicine, Experimental
Neurophysiology
Permeability
Physiological aspects
France
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Summary: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 central nervous system in this mouse model and possibly other models of mitochondrial dysfunction require the use of specific tools to bypass the BBB. In addition, they raise the need for testing the integrity of the BBB in complementary in vivo models.
ISSN:1422-0067
DOI:10.3390/ijms25094828