{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T02:15:27Z","timestamp":1791598527256,"version":"4.3.4"},"reference-count":51,"publisher":"Oxford University Press (OUP)","issue":"24","license":[{"start":{"date-parts":[[2020,12,15]],"date-time":"2020-12-15T00:00:00Z","timestamp":1607990400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Deutsche Muskelstiftung"},{"name":"SMA Europe"},{"name":"Hacettepe University Scientific Projects Coordination Unit","award":["THD-2017-12939"],"award-info":[{"award-number":["THD-2017-12939"]}]},{"name":"Hacettepe University Scientific Projects Coordination Unit","award":["TBI-2015-7546"],"award-info":[{"award-number":["TBI-2015-7546"]}]},{"name":"Scientific and Technological Research Council of Turkey-TUB\u0130TAK","award":["216S770"],"award-info":[{"award-number":["216S770"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,2,25]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Spinal muscular atrophy (SMA) is a devastating childhood disease primarily affecting lower motoneurons in the spinal cord. SMA is caused by the loss of functional survival of motoneuron (SMN) protein, leading to structural and functional alterations of the cytoskeleton in motoneurons and other cells. Loss of SMN results in impairments of microtubule architecture, but the underlying mechanisms are not completely understood. In this study, we mechanistically analyzed the effects of SMN deficiency on microtubules, demonstrating a reduced stability together with a reduction in alpha tubulin detyrosination. This was caused by increased levels of microtubule-associated protein 1B and tubulin tyrosine ligase, resulting in mitochondrial mislocalization in SMA. Our findings suggest that altered tubulin post-translational modifications and microtubule-associated proteins are involved in the pathomechanisms of SMA, such as an impaired axonal transport of mitochondria.<\/jats:p>","DOI":"10.1093\/hmg\/ddaa275","type":"journal-article","created":{"date-parts":[[2020,12,18]],"date-time":"2020-12-18T17:44:08Z","timestamp":1608313448000},"page":"3935-3944","source":"Crossref","is-referenced-by-count":26,"title":["Microtubule-associated protein 1B dysregulates microtubule dynamics and neuronal mitochondrial transport in spinal muscular atrophy"],"prefix":"10.1093","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4206-8332","authenticated-orcid":false,"given":"Gamze","family":"Bora","sequence":"first","affiliation":[{"name":"Department of Medical Biology, Faculty of Medicine, Hacettepe University, Ankara 06100, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niko","family":"Hensel","sequence":"additional","affiliation":[{"name":"Institute of Neuroanatomy and Cell Biology, OE 4140, Hannover Medical School, Hannover 30625, Germany"},{"name":"Center for Systems Neuroscience (ZSN), Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Rademacher","sequence":"additional","affiliation":[{"name":"Institute of Neuroanatomy and Cell Biology, OE 4140, Hannover Medical School, Hannover 30625, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dila","family":"Koyuno\u011flu","sequence":"additional","affiliation":[{"name":"Department of Medical Biology, Faculty of Medicine, Hacettepe University, Ankara 06100, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Merve","family":"Sunguro\u011flu","sequence":"additional","affiliation":[{"name":"Department of Medical Biology, Faculty of Medicine, Hacettepe University, Ankara 06100, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evrim","family":"Aksu-Menge\u015f","sequence":"additional","affiliation":[{"name":"Department of Medical Biology, Faculty of Medicine, Hacettepe University, Ankara 06100, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Burcu","family":"Balc\u0131-Hayta","sequence":"additional","affiliation":[{"name":"Department of Medical Biology, Faculty of Medicine, Hacettepe University, Ankara 06100, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Claus","sequence":"additional","affiliation":[{"name":"Institute of Neuroanatomy and Cell Biology, OE 4140, Hannover Medical School, Hannover 30625, Germany"},{"name":"Center for Systems Neuroscience (ZSN), Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hayat","family":"Erdem-Yurter","sequence":"additional","affiliation":[{"name":"Department of Medical Biology, Faculty of Medicine, Hacettepe University, Ankara 06100, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,1,7]]},"reference":[{"key":"2021022522085644600_ref1","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1016\/j.ncl.2015.07.004","article-title":"Spinal muscular atrophy","volume":"33","author":"Kolb","year":"2015","journal-title":"Neurol. 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