Zobrazeno 1 - 10 of 68 pro vyhledávání: '"Guillermo Guenechea"'
1
Akademický článek
The gray boundaries of aberrant shortening of the cellular timekeepers’ edges
Autor: Guillermo Guenechea, Nestor W Meza
Publikováno v: EMBO Molecular Medicine, Vol 16, Iss 10, Pp 2276-2278 (2024)
Telomeres are supramolecular structures that allow the DNA strand to fold back on itself and protect the linear chromosome end from being sensed as a double-strand DNA break. Telomeric conservation relies on mechanisms that replace terminal DNA seque
Externí odkaz: https://doaj.org/article/d577c572aeb94d68b2ccba4b908b14c4
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2
Akademický článek
Generation of dyskeratosis congenita-like hematopoietic stem cells through the stable inhibition of DKC1
Autor: Carlos Carrascoso-Rubio, Hidde A. Zittersteijn, Laura Pintado-Berninches, Beatriz Fernández-Varas, M. Luz Lozano, Cristina Manguan-Garcia, Leandro Sastre, Juan A. Bueren, Rosario Perona, Guillermo Guenechea
Publikováno v: Stem Cell Research & Therapy, Vol 12, Iss 1, Pp 1-8 (2021)
Abstract Dyskeratosis congenita (DC) is a rare telomere biology disorder, which results in different clinical manifestations, including severe bone marrow failure. To date, the only curative treatment for the bone marrow failure in DC patients is all
Externí odkaz: https://doaj.org/article/3e9b4086c5e84d35a41e8439e2fd5480
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3
Akademický článek
Generation of a High Number of Healthy Erythroid Cells from Gene-Edited Pyruvate Kinase Deficiency Patient-Specific Induced Pluripotent Stem Cells
Autor: Zita Garate, Oscar Quintana-Bustamante, Ana M. Crane, Emmanuel Olivier, Laurent Poirot, Roman Galetto, Penelope Kosinski, Collin Hill, Charles Kung, Xabi Agirre, Israel Orman, Laura Cerrato, Omaira Alberquilla, Fatima Rodriguez-Fornes, Noemi Fusaki, Felix Garcia-Sanchez, Tabita M. Maia, Maria L. Ribeiro, Julian Sevilla, Felipe Prosper, Shengfang Jin, Joanne Mountford, Guillermo Guenechea, Agnes Gouble, Juan A. Bueren, Brian R. Davis, Jose C. Segovia
Publikováno v: Stem Cell Reports, Vol 5, Iss 6, Pp 1053-1066 (2015)
Pyruvate kinase deficiency (PKD) is a rare erythroid metabolic disease caused by mutations in the PKLR gene. Erythrocytes from PKD patients show an energetic imbalance causing chronic non-spherocytic hemolytic anemia, as pyruvate kinase defects impai
Externí odkaz: https://doaj.org/article/c4e6f66da3a7499d96a1b9ff98b4f2e1
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4
Targeted gene therapy into a safe harbor site in human hematopoietic progenitor cells
Autor: Fátima Rodríguez-Fornés, Oscar Quintana-Bustamante, M. Luz Lozano, Guillermo Guenechea, Juan A. Bueren, José C. Segovia
Publikováno v: Gene Therapy. 27:435-450
Directed gene therapy mediated by nucleases has become a new alternative to lead targeted integration of therapeutic genes in specific regions in the genome. In this work, we have compared the efficiency of two nuclease types, TALEN and meganucleases
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::78f113614f5f5f693fcb7fa049952056
https://doi.org/10.1038/s41434-020-0144-x
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5
Generation of dyskeratosis congenita-like hematopoietic stem cells through the stable inhibition of DKC1
Autor: M. Luz Lozano, Juan A. Bueren, Hidde A. Zittersteijn, Guillermo Guenechea, Cristina Manguan-García, Carlos Carrascoso-Rubio, Leandro Sastre, Beatriz Fernández-Varas, Rosario Perona, Laura Pintado-Berninches
Publikováno v: Digital.CSIC. Repositorio Institucional del CSIC
instname
Stem Cell Research & Therapy
Stem Cell Research & Therapy, Vol 12, Iss 1, Pp 1-8 (2021)
© The Author(s).
Dyskeratosis congenita (DC) is a rare telomere biology disorder, which results in different clinical manifestations, including severe bone marrow failure. To date, the only curative treatment for the bone marrow failure in DC p
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::17f87e5e89b71083d4874e777a39bb3b
https://doi.org/10.1186/s13287-021-02145-8
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6
GSE4-loaded nanoparticles a potential therapy for lung fibrosis that enhances pneumocyte growth, reduces apoptosis and DNA damage
Autor: Lurdes Planas, Beatriz Fernández-Varas, Rosa Guerrero-López, Guillermo Guenechea, Rosario Perona, Elena G Arias-Salgado, Julio Cortijo, Leandro Sastre, Manoli Igartua, Susana P. Egusquiaguirre, Rosa Maria Hernandez, Laura Pintado-Berninches, Cristina Manguan-García, Ana Montes-Worboys, Laura Iarriccio, Maria Molina-Molina, José Luis Pedraz, Adela Serrano
Publikováno v: Digital.CSIC. Repositorio Institucional del CSIC
instname
Addi: Archivo Digital para la Docencia y la Investigación
Universidad del País Vasco
Addi. Archivo Digital para la Docencia y la Investigación
Dipòsit Digital de la UB
Universidad de Barcelona
© 2021 The Authors.
Idiopathic pulmonary fibrosis is a lethal lung fibrotic disease, associated with aging with a mean survival of 2-5 years and no curative treatment. The GSE4 peptide is able to rescue cells from senescence, DNA and oxidative
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f4b95ee43ca3d2eb91ecfadda3332f63
https://pubmed.ncbi.nlm.nih.gov/33638895
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7
Targeted gene therapy into a safe harbor site in human hematopoietic progenitor cells
Autor: Fatima, Rodriguez-Fornes, Oscar, Quintana-Bustamante, M Luz, Lozano, Jose C, Segovia, Juan A, Bueren, Guillermo, Guenechea
Publikováno v: Gene therapy. 27(9)
Directed gene therapy mediated by nucleases has become a new alternative to lead targeted integration of therapeutic genes in specific regions in the genome. In this work, we have compared the efficiency of two nuclease types, TALEN and meganucleases
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::8de315229800a69b8d4eb246511d34fa
https://pubmed.ncbi.nlm.nih.gov/32218505
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9
GSE4 peptide suppresses oxidative and telomere deficiencies in Ataxia Telangiectasia patient cells
Autor: Almudena Serrano-Benitez, Carlos Benitez-Buelga, Elena G Arias-Salgado, Laura Iarriccio, Susana P. Egusquiaguirre, Jaime Carrillo, J.L. Pedraz, Guillermo Guenechea, Rosa Maria Hernandez, Laura Pintado-Berninches, Beatriz Fernández-Varas, Rosario Perona, Cristina Manguan-García, Leandro Sastre, Felipe Cortés-Ledesma, Manoli Igartua
Publikováno v: Digital.CSIC. Repositorio Institucional del CSIC
instname
Cell Death Differ
Ataxia telangiectasia (AT) is a genetic disease caused by mutations in the ATM gene but the mechanisms underlying AT are not completely understood. Key functions of the ATM protein are to sense and regulate cellular redox status and to transduce DNA
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a4213a2488c41187a1d68edd2bd66282
http://hdl.handle.net/10261/202585
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10
Successful engraftment of gene-corrected hematopoietic stem cells in non-conditioned patients with Fanconi anemia
Autor: R Salgado, María L. Lamana, Raquel Hladun, Lara Álvarez, Manfred Schmidt, Laura Cerrato, Julián Sevilla, Jonathan D. Schwartz, Cristina Díaz de Heredia, Juan A. Bueren, Eva Merino, José A. Casado, Paula Río, Anne Galy, M. Luz Lozano, Albert Català, Omaira Alberquilla, Eva M. Galvez, Yari Giménez, Nagore García de Andoín, Anna Raimbault, Rosa Yañez, Roser Pujol, José C. Segovia, Wei Wang, Massimo Bogliolo, Ricardo López, Susana Navarro, Ning Wu, Jordi Barquinero, Guillermo Guenechea, Irina Giralt, Jean Soulier, Jordi Surrallés, Miriam Hernando, Francisco J Roman-Rodriguez, Rebeca Sanchez-Dominguez
Publikováno v: NATURE MEDICINE
r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
instname
r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau
Nature Medicine
Nature Medicine, 2019, 25, pp.1396-1401. ⟨10.1038/s41591-019-0550-z⟩
r-FSJD: Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
Fundació Sant Joan de Déu
Fanconi anemia (FA) is a DNA repair syndrome generated by mutations in any of the 22 FA genes discovered to date1,2. Mutations in FANCA account for more than 60% of FA cases worldwide3,4. Clinically, FA is associated with congenital abnormalities and
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d2b8f8e3a8b6e38248994a628ce12dd6
http://fundanet.fsjd.org/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=16624
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