A Human iPS Cell-Derived Artificial Skeletal Muscle for Regenerative Medicine, Disease Modelling and Drug Screening

Información del proyecto

HISTOID

Identificador del acuerdo de subvención: 759108

DOI

10.3030/759108

Proyecto cerrado

Fecha de la firma de la CE 7 Agosto 2018

Fecha de inicio 1 Septiembre 2018

Fecha de finalización 28 Febrero 2025

Financiado con arreglo a

• EXCELLENT SCIENCE - European Research Council (ERC)

Coste total

€ 1 499 738,00

Aportación de la UE

€ 1 499 738,00

Coordinado por

UNIVERSITY COLLEGE LONDON
United Kingdom

CORDIS proporciona enlaces a los documentos públicos y las publicaciones de los proyectos de los programas marco HORIZONTE.

Los enlaces a los documentos y las publicaciones de los proyectos del Séptimo Programa Marco, así como los enlaces a algunos tipos de resultados específicos, como conjuntos de datos y «software», se obtienen dinámicamente de OpenAIRE .

Publicaciones

Other (3)

DLL4 and PDGF-BB regulate migration of human iPSC-derived skeletal myogenic progenitors

Autores: Giulia Ferrari, SungWoo Choi, Louise A. Moyle, Mackinlay, Naira Naouar, Christine Wells, Francesco Muntoni, Francesco Saverio Tedesco
Publicado en: Biorxiv, 2021
Editor: Cold Spring Harbor Laboratory
DOI: 10.1101/2021.02.28.431778

CRISPR-Cas9 mediated endogenous utrophin upregulation improves Duchenne Muscular Dystrophy

Autores: Simon Guiraud, Sumitava Dastidar, Fetta Mazed, Fatima Amor, Maelle Ralu, Anne de Cian, Isabelle Richard, Giuseppe Ronzitti, Francesco Saverio Tedesco, Mario Amendola
Publicado en: 2023
Editor: Cold Spring Harbor Laboratory
DOI: 10.1101/2023.04.18.536394

Advanced human iPSC-based modelling of <i>LMNA</i> -related congenital muscular dystrophy enables development of targeted genetic therapies for muscle laminopathies

Autores: Daniel P. Moore, Heather B. Steele-Stallard, Luca Pinton, Valentina Maria Lionello, Lucia Rossi, Artadokht Aghaeipour, Salma Jalal, Cherry Tsz Yan Wong, Angela Clara-Hwang, Gisèle Bonne, Peter S. Zammit, Francesco Saverio Tedesco
Publicado en: BioRxiv, 2025
Editor: Cold Spring Harbor Laboratory
DOI: 10.1101/2025.06.22.660928

Peer reviewed articles (16)

3D human induced pluripotent stem cell–derived bioengineered skeletal muscles for tissue, disease and therapy modeling

Autores: Luca Pinton, Moustafa Khedr, Valentina M. Lionello, Shilpita Sarcar, Sara M. Maffioletti, Sumitava Dastidar, Elisa Negroni, SungWoo Choi, Noreen Khokhar, Anne Bigot, John R. Counsell, Andreia Sofia Bernardo, Peter S. Zammit, Francesco Saverio Tedesco
Publicado en: Nature Protocols, Edición 18, 2023, Página(s) 1337-1376, ISSN 1754-2189
Editor: Nature Publishing Group
DOI: 10.1038/s41596-022-00790-8

Metabolic remodeling in hiPSC-derived myofibers carrying the m.3243A&gt;G mutation

Autores: Gabriel E. Valdebenito, Anitta R. Chacko, Chih-Yao Chung, Preethi Sheshadri, Haoyu Chi, Benjamin O'Callaghan, Monika J. Madej, Henry Houlden, Hannah Rouse, Valle Morales, Katiuscia Bianchi, Francesco Saverio Tedesco, Robert D.S. Pitceathly, Michael R. Duchen
Publicado en: Stem Cell Reports, Edición 20, 2025, Página(s) 102448, ISSN 2213-6711
Editor: Cell Press
DOI: 10.1016/j.stemcr.2025.102448

Assessing and enhancing migration of human myogenic progenitors using directed <scp>iPS</scp> cell differentiation and advanced tissue modelling

Autores: SungWoo Choi, Giulia Ferrari, Louise A Moyle, Kirsty Mackinlay, Naira Naouar, Salma Jalal, Sara Benedetti, Christine Wells, Francesco Muntoni, Francesco Saverio Tedesco
Publicado en: EMBO Molecular Medicine, Edición 14, 2023, ISSN 1757-4676
Editor: John Wiley & Sons Ltd.
DOI: 10.15252/emmm.202114526

Generation of a human induced pluripotent stem cell line (CRICKi021-A) from a patient with Ullrich congenital muscular dystrophy carrying a pathogenic mutation in the COL6A1 gene

Autores: Yunsong Jiang, Liani G. Devito, Francesco Muntoni, Lyn Healy, Francesco Saverio Tedesco
Publicado en: Stem Cell Research, Edición 83, 2025, Página(s) 103648, ISSN 1873-5061
Editor: Elsevier
DOI: 10.1016/j.scr.2024.103648

Engineering of human induced pluripotent stem cells via human artificial chromosome vectors for cell therapy and disease modeling

Autores: Yasuhiro Kazuki, Narumi Uno, Satoshi Abe, Naoyo Kajitani, Kanako Kazuki, Yuwna Yakura, Chiaki Sawada, Shuta Takata, Masaki Sugawara, Yuichi Nagashima, Akane Okada, Masaharu Hiratsuka, Mitsuhiko Osaki, Giulia Ferrari, Francesco Saverio Tedesco, Satoshi Nishikawa, Ken Fukumoto, Shin-ichiro Takayanagi, Atsushi Kunisato, Shin Kaneko, Mitsuo Oshimura, Kazuma Tomizuka
Publicado en: Molecular Therapy - Nucleic Acids, Edición 23, 2021, Página(s) 629-639, ISSN 2162-2531
Editor: Nature Publishing Group
DOI: 10.1016/j.omtn.2020.12.012

Bioengineering human skeletal muscle models: Recent advances, current challenges and future perspectives

Autores: Yunsong Jiang, Tugce Torun, Sara M. Maffioletti, Andrea Serio, Francesco Saverio Tedesco
Publicado en: Experimental Cell Research, Edición 416, 2024, Página(s) 113133, ISSN 0014-4827
Editor: Academic Press
DOI: 10.1016/j.yexcr.2022.113133

Cellular dynamics of myogenic cell migration: molecular mechanisms and implications for skeletal muscle cell therapies

Autores: SungWoo Choi, Giulia Ferrari, Francesco Saverio Tedesco
Publicado en: EMBO Molecular Medicine, Edición 12/12, 2020, ISSN 1757-4676
Editor: John Wiley & Sons Ltd.
DOI: 10.15252/emmm.202012357

Generation of an MTM1-mutant iPSC line (CRICKi008-A) from an individual with X-linked myotubular myopathy (XLMTM)

Autores: Liani G. Devito, Valentina M. Lionello, Francesco Muntoni, Francesco Saverio Tedesco, Lyn Healy
Publicado en: Stem Cell Research, Edición 69, 2024, Página(s) 103079, ISSN 1873-5061
Editor: Elsevier
DOI: 10.1016/j.scr.2023.103079

Modeling Skeletal Muscle Laminopathies Using Human Induced Pluripotent Stem Cells Carrying Pathogenic LMNA Mutations

Autores: Heather B. Steele-Stallard, Luca Pinton, Shilpita Sarcar, Tanel Ozdemir, Sara M. Maffioletti, Peter S. Zammit, Francesco Saverio Tedesco
Publicado en: Frontiers in Physiology, Edición 9, 2018, ISSN 1664-042X
Editor: Frontiers Research Foundation
DOI: 10.3389/fphys.2018.01332

Low-cost, versatile, and highly reproducible microfabrication pipeline to generate 3D-printed customised cell culture devices with complex designs

Autores: Cathleen Hagemann, Matthew C. D. Bailey, Eugenia Carraro, Ksenia S. Stankevich, Valentina Maria Lionello, Noreen Khokhar, Pacharaporn Suklai, Carmen Moreno-Gonzalez, Kelly O’Toole, George Konstantinou, Christina L. Dix, Sudeep Joshi, Eleonora Giagnorio, Mads S. Bergholt, Christopher D. Spicer, Albane Imbert, Francesco Saverio Tedesco, Andrea Serio
Publicado en: PLOS Biology, Edición 22, 2024, Página(s) e3002503, ISSN 1545-7885
Editor: PLOS
DOI: 10.1371/journal.pbio.3002503

Ammonia transporter RhBG initiates downstream signaling and functional responses by activating NFκB

Autores: Saurabh Mishra, Nicole Welch, Shashi Shekhar Singh, Khuraijam Dhanachandra Singh, Annette Bellar, Avinash Kumar, Lars N. Deutz, Maxmillian D. Hanlon, Sashi Kant, Sumitava Dastidar, Hailee Patel, Vandana Agrawal, Amy H. Attaway, Ryan Musich, George R. Stark, Francesco Saverio Tedesco, George A. Truskey, I. David Weiner, Sadashiva S. Karnik, Srinivasan Dasarathy
Publicado en: Proceedings of the National Academy of Sciences, Edición 121, 2024, ISSN 0027-8424
Editor: National Academy of Sciences
DOI: 10.1073/pnas.2314760121

Generation of two genomic-integration-free DMD iPSC lines with mutations affecting all dystrophin isoforms and potentially amenable to exon-skipping

Autores: Giulia Ferrari, Francesco Muntoni, Francesco Saverio Tedesco
Publicado en: Stem Cell Research, Edición 43, 2020, Página(s) 101688, ISSN 1873-5061
Editor: Elsevier
DOI: 10.1016/j.scr.2019.101688

Investigating the role of dystrophin isoform deficiency in motor function in Duchenne muscular dystrophy

Autores: Mary Chesshyre, Deborah Ridout, Yasumasa Hashimoto, Yoko Ookubo, Silvia Torelli, Kate Maresh, Valeria Ricotti, Lianne Abbott, Vandana Ayyar Gupta, Marion Main, Giulia Ferrari, Anna Kowala, Yung‐Yao Lin, Francesco Saverio Tedesco, Mariacristina Scoto, Giovanni Baranello, Adnan Manzur, Yoshitsugu Aoki, Francesco Muntoni
Publicado en: Journal of Cachexia, Sarcopenia and Muscle, Edición 13, 2023, Página(s) 1360-1372, ISSN 2190-5991
Editor: Springer Verlag
DOI: 10.1002/jcsm.12914

Advanced models of human skeletal muscle differentiation, development and disease: Three-dimensional cultures, organoids and beyond

Autores: Salma Jalal, Sumitava Dastidar, Francesco Saverio Tedesco
Publicado en: Current Opinion in Cell Biology, Edición 73, 2023, Página(s) 92-104, ISSN 0955-0674
Editor: Elsevier BV
DOI: 10.1016/j.ceb.2021.06.004

Combined Notch and PDGF Signaling Enhances Migration and Expression of Stem Cell Markers while Inducing Perivascular Cell Features in Muscle Satellite Cells

Autores: Mattia Francesco Maria Gerli, Louise Anne Moyle, Sara Benedetti, Giulia Ferrari, Ekin Ucuncu, Martina Ragazzi, Chrystalla Constantinou, Irene Louca, Hiroshi Sakai, Pierpaolo Ala, Paolo De Coppi, Shahragim Tajbakhsh, Giulio Cossu, Francesco Saverio Tedesco
Publicado en: Stem Cell Reports, Edición 12/3, 2019, Página(s) 461-473, ISSN 2213-6711
Editor: Cell Press
DOI: 10.1016/j.stemcr.2019.01.007

Dystrophin deficiency affects human astrocyte properties and response to damage

Autores: Jenny Lange, Olivia Gillham, Reem Alkharji, Simon Eaton, Giulia Ferrari, Monika Madej, Michael Flower, Francesco Saverio Tedesco, Francesco Muntoni, Patrizia Ferretti
Publicado en: Glia, Edición 70, 2023, Página(s) 466-490, ISSN 0894-1491
Editor: John Wiley & Sons Inc.
DOI: 10.1002/glia.24116

Book chapters (1)

Pericytes in Muscular Dystrophies

Autores: Louise Anne Moyle, Francesco Saverio Tedesco, Sara Benedetti
Publicado en: Pericyte Biology in Disease, Edición 1147, 2019, Página(s) 319-344, ISBN 978-3-030-16907-7
Editor: Springer International Publishing
DOI: 10.1007/978-3-030-16908-4_15

Non-peer reviewed articles (2)

Challenging the “chromatin hypothesis” of cardiac laminopathies with LMNA mutant iPS cells

Autores: Chiara Mozzetta, Francesco Saverio Tedesco
Publicado en: Journal of Cell Biology, Edición 218/9, 2019, Página(s) 2826-2828, ISSN 0021-9525
Editor: Rockefeller University Press
DOI: 10.1083/jcb.201907166

Stem cell culture conditions and stability: a joint workshop of the PluriMes Consortium and Pluripotent Stem Cell Platform

Autores: Glyn N Stacey, Peter W Andrews, Ivana Barbaric, Charlotta Boiers, Amit Chandra, Giulio Cossu, Lynn Csontos, Thomas JR Frith, Jason A Halliwell, Zoe Hewitt, Mark McCall, Harry D Moore, Malin Parmar, M Beatrice Panico, Venkat Pisupati, Valentin P Shichkin, Alison R Stacey, Francesco S Tedesco, Oliver Thompson, Ravenska Wagey
Publicado en: Regenerative Medicine, Edición 14/3, 2019, Página(s) 243-255, ISSN 1746-0751
Editor: Future Medicine Ltd.
DOI: 10.2217/rme-2019-0001