mTOR signaling in proteostasis and its relevance to autism spectrum disorders

Mostra el registre complet Registre parcial de l'ítem

• dc.contributor.author Faus-Garriga, Juditca
• dc.contributor.author Novoa, Isabelca
• dc.contributor.author Ozaita Mintegui, Andrés, 1969-ca
• dc.date.accessioned 2017-07-04T07:58:33Z
• dc.date.available 2017-07-04T07:58:33Z
• dc.date.issued 2017
• dc.description.abstract Proteins are extremely labile cellular components, especially at physiological temperatures. The appropriate regulation of protein levels, or proteostasis, is essential for all cells. In the case of highly polarized cells like neurons, proteostasis is also crucial at synapses, where quick confined changes in protein composition occur to support synaptic activity and plasticity. The accurate regulation of those cellular processes controlling protein synthesis and degradation is necessary for proteostasis, and its deregulation has deleterious consequences in brain function. Alterations in those cellular mechanisms supporting synaptic protein homeostasis have been pinpointed in autism spectrum disorders such as tuberous sclerosis, neurofibromatosis 1, PTEN-related disorders, fragile X syndrome, MECP2 disorders and Angelman syndrome. Proteostasis alterations in these disorders share the alterations in mechanistic/mammalian target of rapamycin (mTOR) signaling pathway, an intracellular pathway with key synaptic roles. The aim of the present review is to describe the recent literature on the major cellular mechanisms involved in proteostasis regulation in the synaptic context, and its association with mTOR signaling deregulations in various autism spectrum disorders. Altogether, the cellular and molecular mechanisms in synaptic proteostasis could be the foundation for novel shared therapeutic strategies that would take advantage of targeting common disorder mechanisms.
• dc.description.sponsorship This review was supported by grant BFU2015-68568-P (MINECO/FEDER, EU) to AO.
• dc.format.mimetype application/pdfca
• dc.identifier.citation Faus-Garriga J, Novoa I, Ozaita A. mTOR signaling in proteostasis and its relevance to autism spectrum disorders. AIMS Biophysics. 2017;4(1):63-89. DOI: 10.3934/biophy.2017.1.63
• dc.identifier.doi http://dx.doi.org/10.3934/biophy.2017.1.63
• dc.identifier.issn 2377-9098
• dc.identifier.uri http://hdl.handle.net/10230/32494
• dc.language.iso eng
• dc.publisher AIMS Pressca
• dc.relation.ispartof AIMS Biophysics. 2017;4(1):63-89
• dc.relation.projectID info:eu-repo/grantAgreement/ES/1PE/BFU2015-68568-P
• dc.rights © 2017, Andrés Ozaita, et al., licensee AIMS Press. This is an open access article distributed under the terms of the Creative Commons Attribution Licese (http://creativecommons.org/licenses/by/4.0)
• dc.rights.accessRights info:eu-repo/semantics/openAccess
• dc.rights.uri http://creativecommons.org/licenses/by/4.0
• dc.subject.keyword Proteostasis
• dc.subject.keyword Synaptic function
• dc.subject.keyword Protein translation
• dc.subject.keyword Protein degradation
• dc.subject.keyword Autism spectrum disorders
• dc.subject.keyword MTOR signaling
• dc.title mTOR signaling in proteostasis and its relevance to autism spectrum disordersca
• dc.type info:eu-repo/semantics/article
• dc.type.version info:eu-repo/semantics/publishedVersion