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Your search keyword '"Molinos-Quintana A"' showing total 6 results
Start Over Author "Molinos-Quintana A" Publication Type Magazines
6 results on '"Molinos-Quintana A"'

1. Retrospective and Observational Study on CAR-T 19 Bridge Therapies in Pediatric, Adolescents and Young Adults <24 Years with B-Cell Precursor Acute Lymphoblastic Leukemia (BCP-ALL): Experience of the Spanish Hematopoietic Stem Cell Trasplantation and Cellular Therapy Group (GETH-TC)

Author

Galán-Gómez, Víctor, González-Martínez, Berta, Alonso-Saladrigues, Anna, Rives, Susana, Molinos Quintana, Agueda, Perez Simon, Jose Antonio, Herrero-Velasco, Blanca, Kwon, Mi, Barba, Pere, Sánchez-Salinas, Mario, López-Corral, Lucía, Torrent, Anna, Hernani, Rafael, Leon Triana, Odelaisy, and Pérez-Martínez, Antonio

Abstract

INTRODUCTION

Published
2023
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4. Absence of WASp Enhances Hematopoietic and Megakaryocytic Differentiation in a Human Embryonic Stem Cell Model

Author

Toscano, Miguel G, Muñoz, Pilar, Sánchez-Gilabert, Almudena, Cobo, Marién, Benabdellah, Karim, Anderson, Per, Ramos-Mejía, Verónica, Real, Pedro J, Neth, Olaf, Molinos-Quintana, Agueda, Gregory, Philip D, Holmes, Michael C, and Martin, Francisco

Abstract

The Wiskott-Aldrich syndrome (WAS) is an X-linked primary immunodeficiency caused by mutations in the WASgene and characterized by severe thrombocytopenia. Although the role of WASp in terminally differentiated lymphocytes and myeloid cells is well characterized, its role in early hematopoietic differentiation and in platelets (Plts) biology is poorly understood. In the present manuscript, we have used zinc finger nucleases targeted to the WASlocus for the development of two isogenic WASknockout (WASKO) human embryonic stem cell lines (hESCs). Upon hematopoietic differentiation, hESCs-WASKO generated increased ratios of CD34+CD45+progenitors with altered responses to stem cell factor compared to hESCs-WT. When differentiated toward the megakaryocytic linage, hESCs-WASKO produced increased numbers of CD34+CD41+progenitors, megakaryocytes (MKs), and Plts. hESCs-WASKO-derived MKs and Plts showed altered phenotype as well as defective responses to agonist, mimicking WAS patients MKs and Plts defects. Interestingly, the defects were more evident in WASp-deficient MKs than in WASp-deficient Plts. Importantly, ectopic WASexpression using lentiviral vectors restored normal Plts development and MKs responses. These data validate the AND-1_WASKO cell lines as a human cellular model for basic research and for preclinical studies for WAS.

Published
2016
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