{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T18:51:42Z","timestamp":1791658302001,"version":"4.3.4"},"reference-count":34,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T00:00:00Z","timestamp":1761350400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003329","name":"Spanish Ministry of Economy and Competitiveness","doi-asserted-by":"publisher","award":["SAF2016-79668-R"],"award-info":[{"award-number":["SAF2016-79668-R"]}],"id":[{"id":"10.13039\/501100003329","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004837","name":"Spanish Ministry of Science and Innovation","doi-asserted-by":"publisher","award":["PID2019-106943RB-I00"],"award-info":[{"award-number":["PID2019-106943RB-I00"]}],"id":[{"id":"10.13039\/501100004837","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004837","name":"Spanish Ministry of Science and Innovation","doi-asserted-by":"publisher","award":["PID2022-140456NB-I00"],"award-info":[{"award-number":["PID2022-140456NB-I00"]}],"id":[{"id":"10.13039\/501100004837","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008431","name":"Regional Government of Castile and Leon","doi-asserted-by":"publisher","award":["SA030P17"],"award-info":[{"award-number":["SA030P17"]}],"id":[{"id":"10.13039\/501100008431","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008431","name":"Regional Government of Castile and Leon","doi-asserted-by":"publisher","award":["SA129P20"],"award-info":[{"award-number":["SA129P20"]}],"id":[{"id":"10.13039\/501100008431","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008431","name":"Regional Government of Castile and Leon","doi-asserted-by":"publisher","award":["SA112P24"],"award-info":[{"award-number":["SA112P24"]}],"id":[{"id":"10.13039\/501100008431","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Centre for Regenerative Medicine and Cell Therapy of Castile and Leon"},{"name":"University of Salamanca"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>The cytoskeleton relies heavily on the dynamic nature of microtubules, regulated by post-translational modifications such as polyglutamylation and deglutamylation. Disruption of its internal balance, particularly through the absence of cytosolic carboxypeptidase 1 (CCP1), leads to cytoskeletal collapse and cell death. An example of this occurrence exists in the Purkinje Cell Degeneration (PCD) mouse, a direct animal model for childhood-onset neurodegeneration with cerebellar atrophy (CONDCA) human disease. Both CONDCA patients and PCD mice suffer a dramatic degeneration of Purkinje cells. Intriguingly, lobe X appears less vulnerable to this insult. This study revealed in wild-type mice that lobe X expresses less Ccp1 compared to other lobes, correlating with its delayed degeneration in PCD mice. Further expression analysis of other deglutamylating enzymes (CCP4 and CCP6) and glutamylating enzymes (TTLL1) revealed distinctive patterns: Ccp4 showed minimal relevance in cerebellum, while Ccp6 displayed a compensatory increase during critical stages. Meanwhile, Ttll1 expression remained consistent across lobes, suggesting that the resistance of lobe X may be related to a more dynamic, hyperglutamylated cytoskeleton. Unraveling the neuroresistance mechanisms of Purkinje cells may help mitigate neuronal loss in CONDCA patients and may offer a glimmer of hope for alleviating the symptoms of other neurodegenerative diseases.<\/jats:p>","DOI":"10.3390\/ijms262110378","type":"journal-article","created":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T00:51:42Z","timestamp":1761526302000},"page":"10378","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Specific Glutamylation Patterns of the Cytoskeleton Confer Neuroresistance to Lobe X of the Cerebellum in a Model of Childhood-Onset Neurodegeneration with Cerebellar Atrophy"],"prefix":"10.3390","volume":"26","author":[{"given":"Carlos","family":"Hern\u00e1ndez-P\u00e9rez","sequence":"first","affiliation":[{"name":"Laboratory of Neuronal Plasticity and Neurorepair, Institute for Neuroscience of Castilla y Le\u00f3n (INCyL), Universidad de Salamanca, C\/Pintor Fernando Gallego 1, E-37007 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5993-5973","authenticated-orcid":false,"given":"Andr\u00e9s A.","family":"Calder\u00f3n-Garc\u00eda","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Radiology, Universidad de Valladolid, Av. Ram\u00f3n y Cajal 7, E-47005 Valladolid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2514-3270","authenticated-orcid":false,"given":"David","family":"P\u00e9rez-Boyero","sequence":"additional","affiliation":[{"name":"Laboratory of Neuronal Plasticity and Neurorepair, Institute for Neuroscience of Castilla y Le\u00f3n (INCyL), Universidad de Salamanca, C\/Pintor Fernando Gallego 1, E-37007 Salamanca, Spain"},{"name":"Institute of Biomedical Research of Salamanca (IBSAL), P.\u00ba de San Vicente, 182, E-37007 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9410-5510","authenticated-orcid":false,"given":"Ver\u00f3nica","family":"Gonz\u00e1lez-N\u00fa\u00f1ez","sequence":"additional","affiliation":[{"name":"Institute of Biomedical Research of Salamanca (IBSAL), P.\u00ba de San Vicente, 182, E-37007 Salamanca, Spain"},{"name":"Department Biochemistry and Molecular Biology, Faculty of Medicine, Universidad de Salamanca, C\/Alfonso X el Sabio, s\/n, E-37007 Salamanca, Spain"},{"name":"Laboratory of Neurochemistry and Molecular Neuroscience, Institute for Neuroscience of Castilla y Le\u00f3n (INCyL), Universidad de Salamanca, C\/Pintor Fernando Gallego 1, E-37007 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2887-5506","authenticated-orcid":false,"given":"Eduardo","family":"Weruaga","sequence":"additional","affiliation":[{"name":"Laboratory of Neuronal Plasticity and Neurorepair, Institute for Neuroscience of Castilla y Le\u00f3n (INCyL), Universidad de Salamanca, C\/Pintor Fernando Gallego 1, E-37007 Salamanca, Spain"},{"name":"Institute of Biomedical Research of Salamanca (IBSAL), P.\u00ba de San Vicente, 182, E-37007 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3705-817X","authenticated-orcid":false,"given":"David","family":"D\u00edaz","sequence":"additional","affiliation":[{"name":"Laboratory of Neuronal Plasticity and Neurorepair, Institute for Neuroscience of Castilla y Le\u00f3n (INCyL), Universidad de Salamanca, C\/Pintor Fernando Gallego 1, E-37007 Salamanca, Spain"},{"name":"Institute of Biomedical Research of Salamanca (IBSAL), P.\u00ba de San Vicente, 182, E-37007 Salamanca, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,10,25]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e100540","DOI":"10.15252\/embj.2018100540","article-title":"Loss of tubulin deglutamylase CCP1 causes infantile-onset neurodegeneration","volume":"37","author":"Shashi","year":"2018","journal-title":"EMBO J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1580","DOI":"10.1002\/ajmg.a.61198","article-title":"Biallelic variant in AGTPBP1 causes infantile lower motor neuron degeneration and cerebellar atrophy","volume":"179","author":"Karakaya","year":"2019","journal-title":"Am. 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