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One of the proteins involved in this system is HERC1 E3 ubiquitin ligase, which was associated with several processes including the normal development and neurotransmission at the neuromuscular junction (NMJ), autophagy in projection neurons, myelination of the peripheral nervous system, among others. The tambaleante (tbl) mouse model carries the spontaneous mutation Gly483Glu substitution in the HERC1 E3 protein. Using this model, we analyzed the implication of HERC1 E3 ubiquitin ligase in the activity of UPS, autophagy, and synaptic homeostasis in brain and muscle tissues. Regarding UPS, no differences were found in its activity nor in the specific gene expression in both brain and muscle tissues from tbl compared with the control littermates. Furthermore, the use of the specific UPS inhibitor (MG-132), did not alter the evoked neurotransmitter release in the levator auris longus (LAL) muscle. Interestingly, the expression of the autophagy-related gene p62 was significantly increased in the muscle of tbl compared to the control littermates. Indeed, impaired evoked neurotransmitter release was observed with the autophagy inhibitor Wortmannin. Finally, altered levels of Clathrin and Synaptophysin were detected in muscle tissues. Altogether, our findings show that HERC1 E3 ubiquitin ligase mutation found in tbl mice alters autophagy and vesicular recycling without affecting proteasomal function.<\/jats:p>","DOI":"10.3390\/ijms26020793","type":"journal-article","created":{"date-parts":[[2025,1,21]],"date-time":"2025-01-21T06:53:32Z","timestamp":1737442412000},"page":"793","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["HERC1 E3 Ubiquitin Ligase Is Necessary for Autophagy Processes and for the Maintenance and Homeostasis of Vesicles in Motor Nerve Terminals, but Not for Proteasomal Activity"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2850-3470","authenticated-orcid":false,"given":"Miguel \u00c1ngel","family":"P\u00e9rez-Castro","sequence":"first","affiliation":[{"name":"Institute of Biomedicine of Seville (IBiS), Virgen del Roc\u00edo University Hospital\/CSIC\/University of Seville, 41013 Seville, Spain"},{"name":"Department of Biochemistry and Molecular Biology, School of Pharmacy, University of Seville, 41012 Seville, Spain"},{"name":"Center for Molecular and Systems Biology, Lunenfeld-Tanenbaum Research Institute, Mount Sinai Hospital, Toronto, ON M5G 1X5, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francisco","family":"Hern\u00e1ndez-Rasco","sequence":"additional","affiliation":[{"name":"Institute of Biomedicine of Seville (IBiS), Virgen del Roc\u00edo University Hospital\/CSIC\/University of Seville, 41013 Seville, Spain"},{"name":"Department of Biochemistry and Molecular Biology, School of Pharmacy, University of Seville, 41012 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7475-8653","authenticated-orcid":false,"given":"Isabel Mar\u00eda","family":"Alonso-Bellido","sequence":"additional","affiliation":[{"name":"Institute of Biomedicine of Seville (IBiS), Virgen del Roc\u00edo University Hospital\/CSIC\/University of Seville, 41013 Seville, Spain"},{"name":"Department of Biochemistry and Molecular Biology, School of Pharmacy, University of Seville, 41012 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda S.","family":"Letr\u00e1n-S\u00e1nchez","sequence":"additional","affiliation":[{"name":"Institute of Biomedicine of Seville (IBiS), Virgen del Roc\u00edo University Hospital\/CSIC\/University of Seville, 41013 Seville, Spain"},{"name":"Department of Biochemistry and Molecular Biology, School of Pharmacy, University of Seville, 41012 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eva Mar\u00eda","family":"P\u00e9rez-Villegas","sequence":"additional","affiliation":[{"name":"Department of Physiology, Anatomy and Cellular Biology, University of Pablo de Olavide, 41013 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5292-1851","authenticated-orcid":false,"given":"Joana","family":"Vitall\u00e9","sequence":"additional","affiliation":[{"name":"Institute of Biomedicine of Seville (IBiS), Virgen del Roc\u00edo University Hospital\/CSIC\/University of Seville, 41013 Seville, Spain"},{"name":"Clinical Unit of Infectious Diseases, Microbiology and Parasitology, Laboratory of Immunovirology, Virgen del Roc\u00edo University Hospital, 41013 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luis Miguel","family":"Real","sequence":"additional","affiliation":[{"name":"Unit of Infectious Diseases and Microbiology, Institute of Biomedicine of Seville (IBiS), University Hospital of Valme\/CSIC\/University of Seville, 41013 Seville, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Infecciosas (CIBERINFEC), 28029 Madrid, Spain"},{"name":"Department of Medical Biochemistry, Molecular Biology and Immunology, School of Medicine, University of Seville, 41009 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6747-7813","authenticated-orcid":false,"given":"Ezequiel","family":"Ruiz-Mateos","sequence":"additional","affiliation":[{"name":"Institute of Biomedicine of Seville (IBiS), Virgen del Roc\u00edo University Hospital\/CSIC\/University of Seville, 41013 Seville, Spain"},{"name":"Clinical Unit of Infectious Diseases, Microbiology and Parasitology, Laboratory of Immunovirology, Virgen del Roc\u00edo University Hospital, 41013 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1137-8706","authenticated-orcid":false,"given":"Jos\u00e9 Luis","family":"Venero","sequence":"additional","affiliation":[{"name":"Institute of Biomedicine of Seville (IBiS), Virgen del Roc\u00edo University Hospital\/CSIC\/University of Seville, 41013 Seville, Spain"},{"name":"Department of Biochemistry and Molecular Biology, School of Pharmacy, University of Seville, 41012 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4027-4044","authenticated-orcid":false,"given":"Luc\u00eda","family":"Tabares","sequence":"additional","affiliation":[{"name":"Department of Medical Physiology and Biophysics, University of Seville, 41009 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u00c1ngel Manuel","family":"Carri\u00f3n","sequence":"additional","affiliation":[{"name":"Department of Physiology, Anatomy and Cellular Biology, University of Pablo de Olavide, 41013 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9452-7828","authenticated-orcid":false,"given":"Jos\u00e9 \u00c1ngel","family":"Armengol","sequence":"additional","affiliation":[{"name":"Department of Physiology, Anatomy and 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Seville (IBiS), Virgen del Roc\u00edo University Hospital\/CSIC\/University of Seville, 41013 Seville, Spain"},{"name":"Department of Biochemistry and Molecular Biology, School of Pharmacy, University of Seville, 41012 Seville, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,1,18]]},"reference":[{"key":"ref_1","first-page":"5","article-title":"The HERC proteins and the nervous system","volume":"132","author":"Ruiz","year":"2021","journal-title":"Semin. 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