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ERBB3 and NGFR mark a distinct skeletal muscle progenitor cell in human development and hPSCs.
Hicks, Michael R; Hiserodt, Julia; Paras, Katrina; Fujiwara, Wakana; Eskin, Ascia; Jan, Majib; Xi, Haibin; Young, Courtney S; Evseenko, Denis; Nelson, Stanley F; Spencer, Melissa J; Handel, Ben Van; Pyle, April D.
Afiliação
  • Hicks MR; Eli and Edythe Broad Center of Regenerative Medicine and Stem Cell Research, University of California, Los Angeles, CA, USA.
  • Hiserodt J; Center for Duchenne Muscular Dystrophy, University of California, Los Angeles, CA, USA.
  • Paras K; Department of Microbiology, Immunology, and Molecular Genetics, University of California, Los Angeles, CA, USA.
  • Fujiwara W; Department of Microbiology, Immunology, and Molecular Genetics, University of California, Los Angeles, CA, USA.
  • Eskin A; Department of Microbiology, Immunology, and Molecular Genetics, University of California, Los Angeles, CA, USA.
  • Jan M; Department of Microbiology, Immunology, and Molecular Genetics, University of California, Los Angeles, CA, USA.
  • Xi H; Center for Duchenne Muscular Dystrophy, University of California, Los Angeles, CA, USA.
  • Young CS; Department of Human Genetics, University of California, Los Angeles, CA, USA.
  • Evseenko D; Eli and Edythe Broad Center of Regenerative Medicine and Stem Cell Research, University of California, Los Angeles, CA, USA.
  • Nelson SF; Center for Duchenne Muscular Dystrophy, University of California, Los Angeles, CA, USA.
  • Spencer MJ; Department of Microbiology, Immunology, and Molecular Genetics, University of California, Los Angeles, CA, USA.
  • Handel BV; Eli and Edythe Broad Center of Regenerative Medicine and Stem Cell Research, University of California, Los Angeles, CA, USA.
  • Pyle AD; Center for Duchenne Muscular Dystrophy, University of California, Los Angeles, CA, USA.
Nat Cell Biol ; 20(1): 46-57, 2018 01.
Article em En | MEDLINE | ID: mdl-29255171
ABSTRACT
Human pluripotent stem cells (hPSCs) can be directed to differentiate into skeletal muscle progenitor cells (SMPCs). However, the myogenicity of hPSC-SMPCs relative to human fetal or adult satellite cells remains unclear. We observed that hPSC-SMPCs derived by directed differentiation are less functional in vitro and in vivo compared to human satellite cells. Using RNA sequencing, we found that the cell surface receptors ERBB3 and NGFR demarcate myogenic populations, including PAX7 progenitors in human fetal development and hPSC-SMPCs. We demonstrated that hPSC skeletal muscle is immature, but inhibition of transforming growth factor-ß signalling during differentiation improved fusion efficiency, ultrastructural organization and the expression of adult myosins. This enrichment and maturation strategy restored dystrophin in hundreds of dystrophin-deficient myofibres after engraftment of CRISPR-Cas9-corrected Duchenne muscular dystrophy human induced pluripotent stem cell-SMPCs. The work provides an in-depth characterization of human myogenesis, and identifies candidates that improve the in vivo myogenic potential of hPSC-SMPCs to levels that are equal to directly isolated human fetal muscle cells.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Receptores de Fator de Crescimento Neural / Fibras Musculares Esqueléticas / Distrofia Muscular de Duchenne / Receptor ErbB-3 / Desenvolvimento Muscular / Mioblastos / Proteínas do Tecido Nervoso Tipo de estudo: Prognostic_studies Limite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Nat Cell Biol Ano de publicação: 2018 Tipo de documento: Article País de afiliação: Estados Unidos
Texto completo
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Receptores de Fator de Crescimento Neural / Fibras Musculares Esqueléticas / Distrofia Muscular de Duchenne / Receptor ErbB-3 / Desenvolvimento Muscular / Mioblastos / Proteínas do Tecido Nervoso Tipo de estudo: Prognostic_studies Limite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Nat Cell Biol Ano de publicação: 2018 Tipo de documento: Article País de afiliação: Estados Unidos