{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T23:12:02Z","timestamp":1789168322420,"version":"build-2803163510"},"reference-count":50,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2021,1,2]],"date-time":"2021-01-02T00:00:00Z","timestamp":1609545600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Mitochondrial dysfunction contributes to neurodegenerative diseases and developmental disorders such as Fragile X syndrome (FXS). The cross-talk between mitochondria and extracellular vesicles (EVs) suggests that EVs may transfer mitochondrial components as intermediators for intracellular communication under physiological and pathological conditions. In the present study, the ability of EVs to transfer mitochondrial components and their role in mitochondrial dysfunction in astrocytes were examined in the brains of Fmr1 knockout (KO) mice, a model of FXS. The amounts of mitochondrial transcription factor NRF-1, ATP synthases ATP5A and ATPB, and the mitochondrial membrane protein VDAC1 in EVs were reduced in cerebral cortex samples and astrocytes from Fmr1 KO mice. These reductions correspond to decreased mitochondrial biogenesis and transcriptional activities in Fmr1 KO brain, along with decreased mitochondrial membrane potential (MMP) with abnormal localization of vimentin intermediate filament (VIF) in Fmr1 KO astrocytes. Our results suggest that mitochondrial dysfunction in astrocytes is associated with the pathogenesis of FXS and can be monitored by depletion of components in EVs. These findings may improve the ability to diagnose developmental diseases associated with mitochondrial dysfunction, such as FXS and autism spectrum disorders (ASD).<\/jats:p>","DOI":"10.3390\/ijms22010410","type":"journal-article","created":{"date-parts":[[2021,1,3]],"date-time":"2021-01-03T19:54:46Z","timestamp":1609703686000},"page":"410","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Depletion of Mitochondrial Components from Extracellular Vesicles Secreted from Astrocytes in a Mouse Model of Fragile X Syndrome"],"prefix":"10.3390","volume":"22","author":[{"given":"Byung Geun","family":"Ha","sequence":"first","affiliation":[{"name":"Research Group of Developmental Disorders and Rare Diseases, Korea Brain Research Institute (KBRI), Daegu 41062, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jung-Yoon","family":"Heo","sequence":"additional","affiliation":[{"name":"Research Group of Developmental Disorders and Rare Diseases, Korea Brain Research Institute (KBRI), Daegu 41062, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5660-3001","authenticated-orcid":false,"given":"Yu-Jin","family":"Jang","sequence":"additional","affiliation":[{"name":"Research Group of Developmental Disorders and Rare Diseases, Korea Brain Research Institute (KBRI), Daegu 41062, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tae-Shin","family":"Park","sequence":"additional","affiliation":[{"name":"Research Group of Developmental Disorders and Rare Diseases, Korea Brain Research Institute (KBRI), Daegu 41062, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ju-Yeon","family":"Choi","sequence":"additional","affiliation":[{"name":"Research Group of Developmental Disorders and Rare Diseases, Korea Brain Research Institute (KBRI), Daegu 41062, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Woo Young","family":"Jang","sequence":"additional","affiliation":[{"name":"Research Group of Developmental Disorders and Rare Diseases, Korea Brain Research Institute (KBRI), Daegu 41062, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sung-Jin","family":"Jeong","sequence":"additional","affiliation":[{"name":"Research Group of Developmental Disorders and Rare Diseases, Korea Brain Research Institute (KBRI), Daegu 41062, Korea"},{"name":"Department of Brain and Cognitive Sciences, Daegu Gyeongbuk Institute of Science and Technology (DGIST), Daegu 42988, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,1,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"190","DOI":"10.25100\/cm.v45i4.1810","article-title":"Fragile X syndrome","volume":"45","author":"Saldarriaga","year":"2014","journal-title":"Colomb. 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