{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T09:25:52Z","timestamp":1772184352576,"version":"3.50.1"},"reference-count":102,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2021,11,25]],"date-time":"2021-11-25T00:00:00Z","timestamp":1637798400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["ANR-2017-CE12-0027."],"award-info":[{"award-number":["ANR-2017-CE12-0027."]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Visual deficit is one of the complications of Huntington disease (HD), a fatal neurological disorder caused by CAG trinucleotide expansions in the Huntingtin gene, leading to the production of mutant Huntingtin (mHTT) protein. Transgenic HD R6\/1 mice expressing human HTT exon1 with 115 CAG repeats recapitulate major features of the human pathology and exhibit a degeneration of the retina. Our aim was to gain insight into the ultrastructure of the pathological HD R6\/1 retina by electron microscopy (EM). We show that the HD R6\/1 retina is enriched with unusual organelles myelinosomes, produced by retinal neurons and glia. Myelinosomes are present in all nuclear and plexiform layers, in the synaptic terminals of photoreceptors, in the processes of retinal neurons and glial cells, and in the subretinal space. In vitro study shows that myelinosomes secreted by human retinal glial M\u00fcller MIO-M1 cells transfected with EGFP-mHTT-exon1 carry EGFP-mHTT-exon1 protein, as revealed by immuno-EM and Western-blotting. Myelinosomes loaded with mHTT-exon1 are incorporated by naive neuronal\/neuroblastoma SH-SY5Y cells. This results in the emergence of mHTT-exon1 in recipient cells. This process is blocked by membrane fusion inhibitor MDL 28170. Conclusion: Incorporation of myelinosomes carrying mHTT-exon1 in recipient cells may contribute to HD spreading in the retina. Exploring ocular fluids for myelinosome presence could bring an additional biomarker for HD diagnostics.<\/jats:p>","DOI":"10.3390\/ijms222312771","type":"journal-article","created":{"date-parts":[[2021,11,30]],"date-time":"2021-11-30T23:22:28Z","timestamp":1638314548000},"page":"12771","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Myelinosome Organelles in the Retina of R6\/1 Huntington Disease (HD) Mice: Ubiquitous Distribution and Possible Role in Disease Spreading"],"prefix":"10.3390","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9036-4489","authenticated-orcid":false,"given":"Marina G.","family":"Yefimova","sequence":"first","affiliation":[{"name":"Laboratoire Signalisation et Transports Ioniques Membranaires, Universit\u00e9 de Poitiers\/CNRS, 1 Rue Georges Bonnet, 86022 Poitiers, France"},{"name":"Sechenov Institute of Evolutionary Physiology and Biochemistry, Russian Academy of Sciences, 44 Pr. Thorez, 194233 St. Petersburg, Russia"},{"name":"Laboratoire de Biologie de la Reproduction-CECOS, Hopital SUD, 16 Bd de Bulgarie, CEDEX, 35000 Rennes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emile","family":"B\u00e9r\u00e9","sequence":"additional","affiliation":[{"name":"Plateforme IMAGE-UP, 1 Rue Georges Bonnet, 86022 Poitiers, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6372-4346","authenticated-orcid":false,"given":"Anne","family":"Cantereau-Becq","sequence":"additional","affiliation":[{"name":"Laboratoire Signalisation et Transports Ioniques Membranaires, Universit\u00e9 de Poitiers\/CNRS, 1 Rue Georges Bonnet, 86022 Poitiers, France"},{"name":"Plateforme IMAGE-UP, 1 Rue Georges Bonnet, 86022 Poitiers, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annie-Claire","family":"Meunier-Balandre","sequence":"additional","affiliation":[{"name":"Laboratoire Signalisation et Transports Ioniques Membranaires, Universit\u00e9 de Poitiers\/CNRS, 1 Rue Georges Bonnet, 86022 Poitiers, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruno","family":"Merceron","sequence":"additional","affiliation":[{"name":"Plateforme IMAGE-UP, 1 Rue Georges Bonnet, 86022 Poitiers, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Agn\u00e8s","family":"Burel","sequence":"additional","affiliation":[{"name":"Plateforme Mric TEM, BIOSIT UMS 34 80, Universit\u00e9 de Rennes 1, 2 Av. Pr. L\u00e9on Bernard, CEDEX, 35043 Rennes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karine","family":"Merienne","sequence":"additional","affiliation":[{"name":"Laboratory of Cognitive and Adaptive Neurosciences (LNCA), University of Strasbourg, 67000 Strasbourg, France"},{"name":"CNRS UMR 7364, 67000 Strasbourg, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C\u00e9lia","family":"Ravel","sequence":"additional","affiliation":[{"name":"Laboratoire de Biologie de la Reproduction-CECOS, Hopital SUD, 16 Bd de Bulgarie, CEDEX, 35000 Rennes, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fr\u00e9d\u00e9ric","family":"Becq","sequence":"additional","affiliation":[{"name":"Laboratoire Signalisation et Transports Ioniques Membranaires, Universit\u00e9 de Poitiers\/CNRS, 1 Rue Georges Bonnet, 86022 Poitiers, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9563-5142","authenticated-orcid":false,"given":"Nicolas","family":"Bourmeyster","sequence":"additional","affiliation":[{"name":"Laboratoire Signalisation et Transports Ioniques Membranaires, Universit\u00e9 de Poitiers\/CNRS, 1 Rue Georges Bonnet, 86022 Poitiers, France"},{"name":"Service de Biochimie, CHU de Poitiers, 1, Rue de la Mil\u00e9trie, 86021 Poitiers, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,11,25]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1038\/nrneurol.2012.227","article-title":"The retina as a window to the brain-from eye research to CNS disorders","volume":"91","author":"London","year":"2013","journal-title":"Nat. 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