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Resultados de la búsqueda125 resultados
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Profilin1 delivery tunes cytoskeleton dynamics towards CNS axon regeneration
Pinto-Costa R; Sousa SC; Leite SC; Nogueira-Rodrigues J; Ferreira da Silva T; Machado D; Marques J; Costa AC; Liz MA; Bartolini F; Brites P; Costell M; Faessler R; Sousa M. Profilin1
(2020). ArticleJournal of Clinical Investigation..
130 - 4, pp. 2024 – 2040, 2020. DOI: https://doi.org/10.1172/JCI125771
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Overexpression of BvHb2, a Class 2 non-symbiotic hemoglobin from sugar beet, confers drought-induced withering resistance and alters iron content in tomato
Gisbert, C., Timoneda, A, Porcel, R., Ros, R., Mulet, J.M
(2020). Article10, 1754; doi:10.3390/agronomy10111754
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Increased Muscleblind levels by chloroquine treatment improve myotonic dystrophy type 1 phenotypes in in vitro and in vivo models
Bargiela A, Sabater-Arcis M, Espinosa-Espinosa J, Zulaica M, Lopez de Munain A, Artero R - 2019 - Proc Natl Acad Sci U S A
(2019). ArticleMyotonic dystrophy type 1 (DM1) is a life-threatening and chronically debilitating neuromuscular disease caused by the expansion of a CTG trinucleotide repeat in the 3' UTR of the DMPK gene. The mutant RNA forms insoluble structures capable of sequestering RNA binding proteins of the Muscleblind-like (MBNL) family, which ultimately leads to phenotypes. In this work, we demonstrate that treatment with the antiautophagic drug chloroquine was sufficient to up-regulate MBNL1 and 2 proteins in Drosophila and mouse (HSALR) models and patient-derived myoblasts. Extra Muscleblind was functional at the molecular level and improved splicing events regulated by MBNLs in all disease models. In vivo,...
Myotonic dystrophy type 1 (DM1) is a life-threatening and chronically debilitating neuromuscular disease caused by the expansion of a CTG trinucleotide repeat in the 3' UTR of the DMPK gene. The mutant RNA forms insoluble structures capable of sequestering RNA binding proteins of the Muscleblind-like (MBNL) family, which ultimately leads to phenotypes. In this work, we demonstrate that treatment with the antiautophagic drug chloroquine was sufficient to up-regulate MBNL1 and 2 proteins in Drosophila and mouse (HSALR) models and patient-derived myoblasts. Extra Muscleblind was functional at the molecular level and improved splicing events regulated by MBNLs in all disease models. In vivo, chloroquine restored locomotion, rescued average cross-sectional muscle area, and extended median survival in DM1 flies. In HSALR mice, the drug restored muscular strength and histopathology signs and reduced the grade of myotonia. Taken together, these results offer a means to replenish critically low MBNL levels in DM1.
Leer más Ocultar DOI: 10.1073/pnas.1820297116 -
Drosophila as a model system for the identification of pharmacological therapies in neurodegenerative diseases. In: Mutsuddi M., Mukherjee A. (eds) Insights into human neurodegeneration: lessons learnt from Drosophila
Solana-Manrique C., Moltó M.D., Calap-Quintana P., Sanz F.J., Llorens J.V., Paricio N.
(2019). LlibreeBook ISBN 978-981-13-2218-1; Hardcover ISBN 978-981-13-2217-4.
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Alterations of perineuronal nets in the dorsolateral prefrontal cortex of neuropsychiatric patients
Alcaide J, Guirado R, Crespo C, Blasco-Ibáñez JM, Varea E, Sanjuan J, Nacher J
(2019). Article7(1):24
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MicroRNA-Based Therapeutic Perspectives in Myotonic Dystrophy
López Castel A, Overby SJ, Artero R - 2019 - Int J Mol Sci
(2019). ArticleMyotonic dystrophy involves two types of chronically debilitating rare neuromuscular diseases: type 1 (DM1) and type 2 (DM2). Both share similarities in molecular cause, clinical signs, and symptoms with DM2 patients usually displaying milder phenotypes. It is well documented that key clinical symptoms in DM are associated with a strong mis-regulation of RNA metabolism observed in patient's cells. This mis-regulation is triggered by two leading DM-linked events: the sequestration of Muscleblind-like proteins (MBNL) and the mis-regulation of the CUGBP RNA-Binding Protein Elav-Like Family Member 1 (CELF1) that cause significant alterations to their important functions in RNA processing. It...
Myotonic dystrophy involves two types of chronically debilitating rare neuromuscular diseases: type 1 (DM1) and type 2 (DM2). Both share similarities in molecular cause, clinical signs, and symptoms with DM2 patients usually displaying milder phenotypes. It is well documented that key clinical symptoms in DM are associated with a strong mis-regulation of RNA metabolism observed in patient's cells. This mis-regulation is triggered by two leading DM-linked events: the sequestration of Muscleblind-like proteins (MBNL) and the mis-regulation of the CUGBP RNA-Binding Protein Elav-Like Family Member 1 (CELF1) that cause significant alterations to their important functions in RNA processing. It has been suggested that DM1 may be treatable through endogenous modulation of the expression of MBNL and CELF1 proteins. In this study, we analyzed the recent identification of the involvement of microRNA (miRNA) molecules in DM and focus on the modulation of these miRNAs to therapeutically restore normal MBNL or CELF1 function. We also discuss additional prospective miRNA targets, the use of miRNAs as disease biomarkers, and additional promising miRNA-based and miRNA-targeting drug development strategies. This review provides a unifying overview of the dispersed data on miRNA available in the context of DM.
Leer más Ocultar DOI: 10.3390/ijms20225600 -
Chronic Stress Modulates Interneuronal Plasticity: Effects on PSA-NCAM and Perineuronal Nets in Cortical and Extracortical Regions
Pesarico AP, Bueno-Fernandez C, Guirado R, Gómez-Climent MÁ, Curto Y, Carceller H, Nacher J
(2019). Article13:197
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The contribution of cannabis use to variation in the incidence of psychotic disorder across Europe (EU-GEI): a multicentre case-control study
Di Forti M, Quattrone D, Freeman TP, Tripoli G, Gayer-Anderson C, Quigley H, Rodriguez V, Jongsma HE, Ferraro L, La Cascia C, La Barbera D, Tarricone I, Berardi D, Szöke A, Arango C, Tortelli A, Velthorst E, Bernardo M, Del-Ben CM, Menezes PR, Selten JP, Jones PB, Kirkbride JB, Rutten BP, de Haan L, Sham PC, van Os J, Lewis CM, Lynskey M, Morgan C, Murray RM
(2019). Article6(5):427-436
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Lack of MeCP2 leads to region-specific increase of doublecortin in the olfactory system
Martínez-Rodríguez E, Martín-Sánchez A, Coviello S, Foiani C, Kul E, Stork O, Martínez-García F, Nacher J, Lanuza E, Santos M, Agustín-Pavón C
(2019). Article224(4):1647-1658
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Young bone marrow transplantation preserves learning and memory in old mice
Das, M.M., Godoy, M., Chen, S., Moser, V.A, Avalos, P., Roxas, K.M., Dang, I., Yáñez, A., Zhang, W., Bresee, C., Arditi, M., Liu, G.Y., Svendsen, C.N., Goodridge, H.S.,
(2019). ArticleCommunications Biology.
2:73, (2019)
Category: “Biology”, Rank: 17/93 (Q1), IF: 4.165 (2019)
