{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T14:54:57Z","timestamp":1787064897444,"version":"build-2736575974"},"reference-count":43,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2024,4,29]],"date-time":"2024-04-29T00:00:00Z","timestamp":1714348800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"AFM-T\u00e9l\u00e9thon","award":["#23650"],"award-info":[{"award-number":["#23650"]}]},{"name":"AFM-T\u00e9l\u00e9thon","award":["#ANR-17-CE37-0008-01"],"award-info":[{"award-number":["#ANR-17-CE37-0008-01"]}]},{"name":"AFM-T\u00e9l\u00e9thon","award":["ANR-15-IDEX-02 NeuroCoG"],"award-info":[{"award-number":["ANR-15-IDEX-02 NeuroCoG"]}]},{"name":"United mitochondrial disease Foundation","award":["#23650"],"award-info":[{"award-number":["#23650"]}]},{"name":"United mitochondrial disease Foundation","award":["#ANR-17-CE37-0008-01"],"award-info":[{"award-number":["#ANR-17-CE37-0008-01"]}]},{"name":"United mitochondrial disease Foundation","award":["ANR-15-IDEX-02 NeuroCoG"],"award-info":[{"award-number":["ANR-15-IDEX-02 NeuroCoG"]}]},{"name":"Agence Nationale de la Recherche","award":["#23650"],"award-info":[{"award-number":["#23650"]}]},{"name":"Agence Nationale de la Recherche","award":["#ANR-17-CE37-0008-01"],"award-info":[{"award-number":["#ANR-17-CE37-0008-01"]}]},{"name":"Agence Nationale de la Recherche","award":["ANR-15-IDEX-02 NeuroCoG"],"award-info":[{"award-number":["ANR-15-IDEX-02 NeuroCoG"]}]},{"name":"Edmond J. Safra philanthropic Foundation","award":["#23650"],"award-info":[{"award-number":["#23650"]}]},{"name":"Edmond J. Safra philanthropic Foundation","award":["#ANR-17-CE37-0008-01"],"award-info":[{"award-number":["#ANR-17-CE37-0008-01"]}]},{"name":"Edmond J. Safra philanthropic Foundation","award":["ANR-15-IDEX-02 NeuroCoG"],"award-info":[{"award-number":["ANR-15-IDEX-02 NeuroCoG"]}]},{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["#23650"],"award-info":[{"award-number":["#23650"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["#ANR-17-CE37-0008-01"],"award-info":[{"award-number":["#ANR-17-CE37-0008-01"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["ANR-15-IDEX-02 NeuroCoG"],"award-info":[{"award-number":["ANR-15-IDEX-02 NeuroCoG"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Inserm","award":["#23650"],"award-info":[{"award-number":["#23650"]}]},{"name":"Inserm","award":["#ANR-17-CE37-0008-01"],"award-info":[{"award-number":["#ANR-17-CE37-0008-01"]}]},{"name":"Inserm","award":["ANR-15-IDEX-02 NeuroCoG"],"award-info":[{"award-number":["ANR-15-IDEX-02 NeuroCoG"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>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\u2013brain 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\u2212\/\u2212 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.<\/jats:p>","DOI":"10.3390\/ijms25094828","type":"journal-article","created":{"date-parts":[[2024,5,1]],"date-time":"2024-05-01T03:30:49Z","timestamp":1714534249000},"page":"4828","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["The Blood\u2013Brain Barrier Is Unaffected in the Ndufs4\u2212\/\u2212 Mouse Model of Leigh Syndrome"],"prefix":"10.3390","volume":"25","author":[{"given":"Robin","family":"Reynaud-Dulaurier","sequence":"first","affiliation":[{"name":"Inserm, U1216, CHU Grenoble Alpes, Grenoble Institut Neurosciences, Universit\u00e9 Grenoble Alpes, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romain","family":"Cl\u00e9ment","sequence":"additional","affiliation":[{"name":"Inserm, U1216, CHU Grenoble Alpes, Grenoble Institut Neurosciences, Universit\u00e9 Grenoble Alpes, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Yjjou","sequence":"additional","affiliation":[{"name":"Inserm, U1216, CHU Grenoble Alpes, Grenoble Institut Neurosciences, Universit\u00e9 Grenoble Alpes, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cassandra","family":"Cresson","sequence":"additional","affiliation":[{"name":"Inserm, U1216, CHU Grenoble Alpes, Grenoble Institut Neurosciences, Universit\u00e9 Grenoble Alpes, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasmina","family":"Saoudi","sequence":"additional","affiliation":[{"name":"Inserm, U1216, CHU Grenoble Alpes, Grenoble Institut Neurosciences, Universit\u00e9 Grenoble Alpes, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathilde","family":"Faideau","sequence":"additional","affiliation":[{"name":"Inserm, U1216, CHU Grenoble Alpes, Grenoble Institut Neurosciences, Universit\u00e9 Grenoble Alpes, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7596-3210","authenticated-orcid":false,"given":"Michael","family":"Decressac","sequence":"additional","affiliation":[{"name":"Inserm, U1216, CHU Grenoble Alpes, Grenoble Institut Neurosciences, Universit\u00e9 Grenoble Alpes, 38000 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,4,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1038\/nrn1824","article-title":"Astrocyte-endothelial interactions at the blood-brain barrier","volume":"7","author":"Abbott","year":"2006","journal-title":"Nat. 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