000078988 001__ 78988 000078988 005__ 20240104111811.0 000078988 0247_ $$2doi$$a10.14336/AD.2018.0917 000078988 0248_ $$2sideral$$a111569 000078988 037__ $$aART-2019-111569 000078988 041__ $$aeng 000078988 100__ $$0(orcid)0000-0001-5193-7782$$aCalvo, Ana C.$$uUniversidad de Zaragoza 000078988 245__ $$aCollagen XIX Alpha 1 improves prognosis in amyotrophic lateral sclerosis 000078988 260__ $$c2019 000078988 5060_ $$aAccess copy available to the general public$$fUnrestricted 000078988 5203_ $$aThe identification of more reliable diagnostic or prognostic biomarkers in age-related neurodegenerative diseases, such as Amyotrophic Lateral Sclerosis (ALS), is urgently needed. The objective in this study was to identify more reliable prognostic biomarkers of ALS mirroring neurodegeneration that could be of help in clinical trials. A total of 268 participants from three cohorts were included in this study. The muscle and blood cohorts were analyzed in two cross-sectional studies, while the serial blood cohort was analyzed in a longitudinal study at 6-monthly intervals. Fifteen target genes and fourteen proteins involved in muscle physiology and differentiation, metabolic processes and neuromuscular junction dismantlement were studied in the three cohorts. In the muscle biopsy cohort, the risk for a higher mortality in an ALS patient that showed high Collagen type XIX, alpha 1 (COL19A1) protein levels and a fast progression of the disease was 70.5% (P < 0.05), while in the blood cohort, this risk was 20% (P < 0.01). In the serial blood cohort, the linear mixed model analysis showed a significant association between increasing COL19A1 gene levels along disease progression and a faster progression during the follow-up period of 24 months (P < 0.05). Additionally, higher COL19A1 levels and a faster progression increased 17.9% the mortality risk (P < 0.01). We provide new evidence that COL19A1 can be considered a prognostic biomarker that could help the selection of homogeneous groups of patients for upcoming clinical trial and may be pointed out as a promising therapeutic target in ALS. 000078988 536__ $$9info:eu-repo/grantAgreement/ES/FIS/PI14-00088$$9info:eu-repo/grantAgreement/ES/FIS/PI17-00491$$9info:eu-repo/grantAgreement/ES/FIS/PI17-00949 000078988 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttp://creativecommons.org/licenses/by/3.0/es/ 000078988 590__ $$a5.402$$b2019 000078988 592__ $$a1.533$$b2019 000078988 591__ $$aGERIATRICS & GERONTOLOGY$$b4 / 51 = 0.078$$c2019$$dQ1$$eT1 000078988 593__ $$aGeriatrics and Gerontology$$c2019$$dQ1 000078988 593__ $$aPathology and Forensic Medicine$$c2019$$dQ1 000078988 593__ $$aNeurology (clinical)$$c2019$$dQ1 000078988 593__ $$aCell Biology$$c2019$$dQ2 000078988 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion 000078988 700__ $$aAtencia Cibreiro, Gabriela 000078988 700__ $$aTorre Merino, Paz 000078988 700__ $$aRoy, Juan F. 000078988 700__ $$aGaliana, Adrián 000078988 700__ $$aJuárez Rufian, Alexandra 000078988 700__ $$aCano, Juan M. 000078988 700__ $$aMartin, Miguel Ángel 000078988 700__ $$0(orcid)0000-0002-7277-4318$$aMoreno, Laura$$uUniversidad de Zaragoza 000078988 700__ $$0(orcid)0000-0001-6199-2953$$aLarrodé, Pilar$$uUniversidad de Zaragoza 000078988 700__ $$aCordero Vázquez, Pilar 000078988 700__ $$aGalan, Lucía 000078988 700__ $$aMora, Jesús 000078988 700__ $$aMuñoz-Blanco, José Luis 000078988 700__ $$0(orcid)0000-0001-8301-6902$$aMuñoz, María J.$$uUniversidad de Zaragoza 000078988 700__ $$0(orcid)0000-0001-5740-0185$$aZaragoza, Pilar$$uUniversidad de Zaragoza 000078988 700__ $$aPegoraro, Elena 000078988 700__ $$aSorarú, Gianni 000078988 700__ $$aMora, Marina 000078988 700__ $$aLunetta, Christian 000078988 700__ $$aPenco, Silvana 000078988 700__ $$aTarlarini, Claudia 000078988 700__ $$aEsteban, Jesús 000078988 700__ $$0(orcid)0000-0001-5687-6704$$aOsta, Rosario$$uUniversidad de Zaragoza 000078988 700__ $$aGarcía Redondo, Alberto 000078988 7102_ $$11001$$2420$$aUniversidad de Zaragoza$$bDpto. Anatom.,Embri.Genét.Ani.$$cÁrea Genética 000078988 7102_ $$11007$$2610$$aUniversidad de Zaragoza$$bDpto. Medicina, Psiqu. y Derm.$$cArea Medicina 000078988 7102_ $$11005$$2315$$aUniversidad de Zaragoza$$bDpto. Farmacología y Fisiolog.$$cÁrea Farmacología 000078988 773__ $$g10, 2 (2019), 278-292$$pAging dis.$$tAging and disease$$x2152-5250 000078988 8564_ $$s914348$$uhttps://zaguan.unizar.es/record/78988/files/texto_completo.pdf$$yVersión publicada 000078988 8564_ $$s139070$$uhttps://zaguan.unizar.es/record/78988/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada 000078988 909CO $$ooai:zaguan.unizar.es:78988$$particulos$$pdriver 000078988 951__ $$a2024-01-04-11:02:13 000078988 980__ $$aARTICLE