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dc.creatorIridoy Zulet, Marinaes_ES
dc.creatorZubiri, Irenees_ES
dc.creatorZelaya Huerta, María Victoriaes_ES
dc.creatorMartínez, Leirees_ES
dc.creatorAusín, Karinaes_ES
dc.creatorLachén Montes, Mercedeses_ES
dc.creatorSantamaría, Enriquees_ES
dc.creatorFernández Irigoyen, Joaquínes_ES
dc.creatorJericó Pascual, Ivonnees_ES
dc.date.accessioned2019-08-14T11:21:26Z
dc.date.available2019-08-14T11:21:26Z
dc.date.issued2019
dc.identifier.issn1661-6596
dc.identifier.urihttps://hdl.handle.net/2454/34339
dc.description.abstract(1) Background: Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are neurodegenerative disorders with an overlap in clinical presentation and neuropathology. Common and differential mechanisms leading to protein expression changes and neurodegeneration in ALS and FTD were studied trough a deep neuroproteome mapping of the spinal cord. (2) Methods: A liquid chromatography tandem mass spectrometry (LC-MS/MS) analysis of the spinal cord from ALS-TAR DNA-binding protein 43 (TDP-43) subjects, ubiquitin-positive frontotemporal lobar degeneration (FTLD-U) subjects and controls without neurodegenerative disease was performed. (3) Results: 281 differentially expressed proteins were detected among ALS versus controls, while 52 proteins were dysregulated among FTLD-U versus controls. Thirty-three differential proteins were shared between both syndromes. The resulting data was subjected to network-driven proteomics analysis, revealing mitochondrial dysfunction and metabolic impairment, both for ALS and FTLD-U that could be validated through the confirmation of expression levels changes of the Prohibitin (PHB) complex. (4) Conclusions: ALS-TDP-43 and FTLD-U share molecular and functional alterations, although part of the proteostatic impairment is region-and disease-specific. We have confirmed the involvement of specific proteins previously associated with ALS (Galectin 2 (LGALS3), Transthyretin (TTR), Protein S100-A6 (S100A6), and Protein S100-A11 (S100A11)) and have shown the involvement of proteins not previously described in the ALS context (Methanethiol oxidase (SELENBP1), Peptidyl-prolyl cis-trans isomerase NIMA-interacting 1 (PIN-1), Calcyclin-binding protein (CACYBP) and Rho-associated protein kinase 2 (ROCK2)). © 2018 by the authors. Licensee MDPI, Basel, Switzerland.en
dc.description.sponsorshipThis research is funded by the Navarre Association of ALS’s patients and relatives (ADELA-Navarra) and the Navarrabiomed-Miguel Servet Foundation, through “Ice Bucket Challenge” campaign donations. M.L.-M. was supported by a pre-doctoral fellowship from the Public University of Navarra (UPNA).en
dc.format.extent24 p.
dc.format.mimetypeapplication/pdfen
dc.language.isoengen
dc.publisherMDPIen
dc.relation.ispartofInternational Journal of Molecular Sciences, 2019, 20, 4en
dc.rights© 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license.en
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.subjectAmyotrophic lateral sclerosis (ALS)en
dc.subjectFrontotemporal dementia (FTD)en
dc.subjectMotor neuronen
dc.subjectProteomicsen
dc.titleNeuroanatomical quantitative proteomics reveals common pathogenic biological routes between amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)en
dc.typeinfo:eu-repo/semantics/articleen
dc.typeArtículo / Artikuluaes
dc.contributor.departmentUniversidad Pública de Navarra. Departamento de Ciencias de la Saludes_ES
dc.contributor.departmentNafarroako Unibertsitate Publikoa. Osasun Zientziak Sailaeu
dc.rights.accessRightsinfo:eu-repo/semantics/openAccessen
dc.rights.accessRightsAcceso abierto / Sarbide irekiaes
dc.identifier.doi10.3390/ijms20010004
dc.relation.publisherversionhttps://doi.org/10.3390/ijms20010004
dc.type.versioninfo:eu-repo/semantics/publishedVersionen
dc.type.versionVersión publicada / Argitaratu den bertsioaes
dc.contributor.funderUniversidad Pública de Navarra / Nafarroako Unibertsitate Publikoaes


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© 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license.
Except where otherwise noted, this item's license is described as © 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license.