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Electron microscopic analysis of the influence of iPSC-derived motor neurons on bioengineered human skeletal muscle tissues.
Nguyen, Christine T; Chávez-Madero, Carolina; Jacques, Erik; Musgrave, Brennen; Yin, Ting; Saraci, Kejzi; Gilbert, Penney M; Stewart, Bryan A.
Afiliación
  • Nguyen CT; Department of Biology, University of Toronto Mississauga, Mississauga, ON, L5L 1C6, Canada.
  • Chávez-Madero C; Department of Cell & Systems Biology, University of Toronto, Toronto, ON, M5S 3G5, Canada.
  • Jacques E; Institute of Biomedical Engineering, University of Toronto, Toronto, ON, M5S 3G9, Canada.
  • Musgrave B; Donnelly Centre, University of Toronto, Toronto, ON, M5S 3E1, Canada.
  • Yin T; Institute of Biomedical Engineering, University of Toronto, Toronto, ON, M5S 3G9, Canada.
  • Saraci K; Donnelly Centre, University of Toronto, Toronto, ON, M5S 3E1, Canada.
  • Gilbert PM; Institute of Biomedical Engineering, University of Toronto, Toronto, ON, M5S 3G9, Canada.
  • Stewart BA; Donnelly Centre, University of Toronto, Toronto, ON, M5S 3E1, Canada.
Cell Tissue Res ; 396(1): 57-69, 2024 Apr.
Article en En | MEDLINE | ID: mdl-38326636
ABSTRACT
3D bioengineered skeletal muscle macrotissues are increasingly important for studies of cell biology and development of therapeutics. Tissues derived from immortalized cells obtained from patient samples, or from pluripotent stem cells, can be co-cultured with motor-neurons to create models of human neuromuscular junctions in culture. In this study, we present foundational work on 3D cultured muscle ultrastructure, with and without motor neurons, which is enabled by the development of a new co-culture platform. Our results show that tissues from Duchenne muscular dystrophy patients are poorly organized compared to tissues grown from healthy donor and that the presence of motor neurons invariably improves sarcomere organization. Electron micrographs show that in the presence of motor neurons, filament directionality, banding patterns, z-disc continuity, and the appearance of presumptive SSR and T-tubule profiles all improve in healthy, DMD-, and iPSC-derived muscle tissue. Further work to identify the underlying defects of DMD tissue disorganization and the mechanisms by which motor neurons support muscle are likely to yield potential new therapeutic approaches for treating patients suffering from Duchenne muscular dystrophy.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Distrofia Muscular de Duchenne / Células Madre Pluripotentes Inducidas Límite: Humans Idioma: En Revista: Cell Tissue Res Año: 2024 Tipo del documento: Article País de afiliación: Canadá Pais de publicación: Alemania
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Distrofia Muscular de Duchenne / Células Madre Pluripotentes Inducidas Límite: Humans Idioma: En Revista: Cell Tissue Res Año: 2024 Tipo del documento: Article País de afiliación: Canadá Pais de publicación: Alemania