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dc.contributor.authorDe Pasquale, Valeria-
dc.contributor.authorMoles, Anna-
dc.contributor.authorPavone, Luigi Michele-
dc.date.accessioned2020-04-28T14:05:56Z-
dc.date.available2020-04-28T14:05:56Z-
dc.date.issued2020-04-15-
dc.identifier.citationCells 9(4): 979 (2020)-
dc.identifier.issn2073-4409-
dc.identifier.urihttp://hdl.handle.net/10261/209444-
dc.description© 2020 by the authors.-
dc.description.abstractCathepsins (CTSs) are ubiquitously expressed proteases normally found in the endolysosomal compartment where they mediate protein degradation and turnover. However, CTSs are also found in the cytoplasm, nucleus, and extracellular matrix where they actively participate in cell signaling, protein processing, and trafficking through the plasma and nuclear membranes and between intracellular organelles. Dysregulation in CTS expression and/or activity disrupts cellular homeostasis, thus contributing to many human diseases, including inflammatory and cardiovascular diseases, neurodegenerative disorders, diabetes, obesity, cancer, kidney dysfunction, and others. This review aimed to highlight the involvement of CTSs in inherited lysosomal storage disorders, with a primary focus to the emerging evidence on the role of CTSs in the pathophysiology of Mucopolysaccharidoses (MPSs). These latter diseases are characterized by severe neurological, skeletal and cardiovascular phenotypes, and no effective cure exists to date. The advance in the knowledge of the molecular mechanisms underlying the activity of CTSs in MPSs may open a new challenge for the development of novel therapeutic approaches for the cure of such intractable diseases.-
dc.description.sponsorshipThis research was funded by “Research Challenges” 2018 R + D+i Project from the Spanish Government to A.M.; A.M.’s salary is funded through a Ramon y Cajal Contract by the Spanish Government.-
dc.publisherMultidisciplinary Digital Publishing Institute-
dc.relation.isversionofPublisher’s version-
dc.rightsopenAccess-
dc.subjectCathepsins-
dc.subjectMucopolysaccharidoses-
dc.subjectLysosomal storage diseases-
dc.subjectTherapy-
dc.titleCathepsins in the Pathophysiology of Mucopolysaccharidoses: New Perspectives for Therapy-
dc.typeartículo-
dc.identifier.doihttp://dx.doi.org/10.3390/cells9040979-
dc.description.peerreviewedPeer reviewed-
dc.relation.publisherversionhttps://doi.org/10.3390/cells9040979-
dc.identifier.e-issn2073-4409-
dc.date.updated2020-04-28T14:05:57Z-
dc.rights.licensehttp://creativecommons.org/licenses/by/4.0/-
dc.contributor.funderMinisterio de Ciencia, Innovación y Universidades (España)-
dc.relation.csic-
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