Systemic Supplementation of Collagen VI by Neonatal Transplantation of iPSC-Derived MSCs Improves Histological Phenotype and Function of Col6-Deficient Model Mice

Collagen VI is distributed in the interstitium and is secreted mainly by mesenchymal stromal cells (MSCs) in skeletal muscle. Mutations in COL6A1-3 genes cause a spectrum of COL6-related myopathies. In this study, we performed a systemic transplantation study of human-induced pluripotent stem cell (...

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Autores principales:	Aya Harada, Megumi Goto, Atsuya Kato, Nana Takenaka-Ninagawa, Akito Tanaka, Satoru Noguchi, Makoto Ikeya, Hidetoshi Sakurai
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	iPS cell mesenchymal stromal cells COL6-related myopathy systemic cell transplantation ullrich congenital muscular dystrophy (UCMD) Biology (General) QH301-705.5
Acceso en línea:	https://doaj.org/article/e107aff9d8cc4e0b97962fd95c16cce6
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spelling	oai:doaj.org-article:e107aff9d8cc4e0b97962fd95c16cce62021-11-30T12:08:44ZSystemic Supplementation of Collagen VI by Neonatal Transplantation of iPSC-Derived MSCs Improves Histological Phenotype and Function of Col6-Deficient Model Mice2296-634X10.3389/fcell.2021.790341https://doaj.org/article/e107aff9d8cc4e0b97962fd95c16cce62021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fcell.2021.790341/fullhttps://doaj.org/toc/2296-634XCollagen VI is distributed in the interstitium and is secreted mainly by mesenchymal stromal cells (MSCs) in skeletal muscle. Mutations in COL6A1-3 genes cause a spectrum of COL6-related myopathies. In this study, we performed a systemic transplantation study of human-induced pluripotent stem cell (iPSC)-derived MSCs (iMSCs) into neonatal immunodeficient COL6-related myopathy model (Col6a1KO/NSG) mice to validate the therapeutic potential. Engraftment of the donor cells and the resulting rescued collagen VI were observed at the quadriceps and diaphragm after intraperitoneal iMSC transplantation. Transplanted mice showed improvement in pathophysiological characteristics compared with untreated Col6a1KO/NSG mice. In detail, higher muscle regeneration in the transplanted mice resulted in increased muscle weight and enlarged myofibers. Eight-week-old mice showed increased muscle force and performed better in the grip and rotarod tests. Overall, these findings support the concept that systemic iMSC transplantation can be a therapeutic option for COL6-related myopathies.Aya HaradaMegumi GotoAtsuya KatoNana Takenaka-NinagawaAkito TanakaSatoru NoguchiMakoto IkeyaHidetoshi SakuraiFrontiers Media S.A.articleiPS cellmesenchymal stromal cellsCOL6-related myopathysystemic cell transplantationullrich congenital muscular dystrophy (UCMD)Biology (General)QH301-705.5ENFrontiers in Cell and Developmental Biology, Vol 9 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	iPS cell mesenchymal stromal cells COL6-related myopathy systemic cell transplantation ullrich congenital muscular dystrophy (UCMD) Biology (General) QH301-705.5
spellingShingle	iPS cell mesenchymal stromal cells COL6-related myopathy systemic cell transplantation ullrich congenital muscular dystrophy (UCMD) Biology (General) QH301-705.5 Aya Harada Megumi Goto Atsuya Kato Nana Takenaka-Ninagawa Akito Tanaka Satoru Noguchi Makoto Ikeya Hidetoshi Sakurai Systemic Supplementation of Collagen VI by Neonatal Transplantation of iPSC-Derived MSCs Improves Histological Phenotype and Function of Col6-Deficient Model Mice
description	Collagen VI is distributed in the interstitium and is secreted mainly by mesenchymal stromal cells (MSCs) in skeletal muscle. Mutations in COL6A1-3 genes cause a spectrum of COL6-related myopathies. In this study, we performed a systemic transplantation study of human-induced pluripotent stem cell (iPSC)-derived MSCs (iMSCs) into neonatal immunodeficient COL6-related myopathy model (Col6a1KO/NSG) mice to validate the therapeutic potential. Engraftment of the donor cells and the resulting rescued collagen VI were observed at the quadriceps and diaphragm after intraperitoneal iMSC transplantation. Transplanted mice showed improvement in pathophysiological characteristics compared with untreated Col6a1KO/NSG mice. In detail, higher muscle regeneration in the transplanted mice resulted in increased muscle weight and enlarged myofibers. Eight-week-old mice showed increased muscle force and performed better in the grip and rotarod tests. Overall, these findings support the concept that systemic iMSC transplantation can be a therapeutic option for COL6-related myopathies.
format	article
author	Aya Harada Megumi Goto Atsuya Kato Nana Takenaka-Ninagawa Akito Tanaka Satoru Noguchi Makoto Ikeya Hidetoshi Sakurai
author_facet	Aya Harada Megumi Goto Atsuya Kato Nana Takenaka-Ninagawa Akito Tanaka Satoru Noguchi Makoto Ikeya Hidetoshi Sakurai
author_sort	Aya Harada
title	Systemic Supplementation of Collagen VI by Neonatal Transplantation of iPSC-Derived MSCs Improves Histological Phenotype and Function of Col6-Deficient Model Mice
title_short	Systemic Supplementation of Collagen VI by Neonatal Transplantation of iPSC-Derived MSCs Improves Histological Phenotype and Function of Col6-Deficient Model Mice
title_full	Systemic Supplementation of Collagen VI by Neonatal Transplantation of iPSC-Derived MSCs Improves Histological Phenotype and Function of Col6-Deficient Model Mice
title_fullStr	Systemic Supplementation of Collagen VI by Neonatal Transplantation of iPSC-Derived MSCs Improves Histological Phenotype and Function of Col6-Deficient Model Mice
title_full_unstemmed	Systemic Supplementation of Collagen VI by Neonatal Transplantation of iPSC-Derived MSCs Improves Histological Phenotype and Function of Col6-Deficient Model Mice
title_sort	systemic supplementation of collagen vi by neonatal transplantation of ipsc-derived mscs improves histological phenotype and function of col6-deficient model mice
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/e107aff9d8cc4e0b97962fd95c16cce6
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Systemic Supplementation of Collagen VI by Neonatal Transplantation of iPSC-Derived MSCs Improves Histological Phenotype and Function of Col6-Deficient Model Mice

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