Myogenic reprogramming of bone marrow derived cells in a W⁴¹Dmd(mdx) deficient mouse model.

Lack of expression of dystrophin leads to degeneration of muscle fibers and infiltration of connective and adipose tissue. Cell transplantation therapy has been proposed as a treatment for intractable muscle degenerative disorders. Several reports have demonstrated the ability of bone-marrow derived...

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Autores principales:	Stuart Walsh, Jens Nygren, Annica Pontén, Stefan Jovinge
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2011
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/acfdbed83045419f84768dd6f676bff6
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spelling	oai:doaj.org-article:acfdbed83045419f84768dd6f676bff62021-11-18T07:33:33ZMyogenic reprogramming of bone marrow derived cells in a W⁴¹Dmd(mdx) deficient mouse model.1932-620310.1371/journal.pone.0027500https://doaj.org/article/acfdbed83045419f84768dd6f676bff62011-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/22140444/?tool=EBIhttps://doaj.org/toc/1932-6203Lack of expression of dystrophin leads to degeneration of muscle fibers and infiltration of connective and adipose tissue. Cell transplantation therapy has been proposed as a treatment for intractable muscle degenerative disorders. Several reports have demonstrated the ability of bone-marrow derived cells (BMDC) to contribute to non-haematopoietic tissues including epithelium, heart, liver, skeletal muscle and brain following transplantation by means of fusion and reprogramming. A key issue is the extent to which fusion and reprogramming can occur in vivo, particularly under conditions of myogenic deterioration.To investigate the therapeutic potential of bone marrow transplantation in monogenetic myopathy, green fluorescent protein-positive (GFP+) bone marrow cells were transplanted into non-irradiated c-kit receptor-deficient (W⁴¹) mdx mice. This model allows BMDC reconstitution in the absence of irradiation induced myeloablation. We provide the first report of BMDC fusion in a W⁴¹Dmd(mdx) deficient mouse model.In the absence of irradiation induced injury, few GFP+ cardiomyocytes and muscle fibres were detected 24 weeks post BMT. It was expected that the frequency of fusion in the hearts of W⁴¹Dmd(mdx) mice would be similar to frequencies observed in infarcted mice. Although, it is clear from this study that individual cardiomyocytes with monogenetic deficiencies can be rescued by fusion, it is as clear that in the absence of irradiation, the formation of stable and reprogrammed fusion hybrids occurs, with the current techniques, at very low levels in non-irradiated recipients.Stuart WalshJens NygrenAnnica PonténStefan JovingePublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 6, Iss 11, p e27500 (2011)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Stuart Walsh Jens Nygren Annica Pontén Stefan Jovinge Myogenic reprogramming of bone marrow derived cells in a W⁴¹Dmd(mdx) deficient mouse model.
description	Lack of expression of dystrophin leads to degeneration of muscle fibers and infiltration of connective and adipose tissue. Cell transplantation therapy has been proposed as a treatment for intractable muscle degenerative disorders. Several reports have demonstrated the ability of bone-marrow derived cells (BMDC) to contribute to non-haematopoietic tissues including epithelium, heart, liver, skeletal muscle and brain following transplantation by means of fusion and reprogramming. A key issue is the extent to which fusion and reprogramming can occur in vivo, particularly under conditions of myogenic deterioration.To investigate the therapeutic potential of bone marrow transplantation in monogenetic myopathy, green fluorescent protein-positive (GFP+) bone marrow cells were transplanted into non-irradiated c-kit receptor-deficient (W⁴¹) mdx mice. This model allows BMDC reconstitution in the absence of irradiation induced myeloablation. We provide the first report of BMDC fusion in a W⁴¹Dmd(mdx) deficient mouse model.In the absence of irradiation induced injury, few GFP+ cardiomyocytes and muscle fibres were detected 24 weeks post BMT. It was expected that the frequency of fusion in the hearts of W⁴¹Dmd(mdx) mice would be similar to frequencies observed in infarcted mice. Although, it is clear from this study that individual cardiomyocytes with monogenetic deficiencies can be rescued by fusion, it is as clear that in the absence of irradiation, the formation of stable and reprogrammed fusion hybrids occurs, with the current techniques, at very low levels in non-irradiated recipients.
format	article
author	Stuart Walsh Jens Nygren Annica Pontén Stefan Jovinge
author_facet	Stuart Walsh Jens Nygren Annica Pontén Stefan Jovinge
author_sort	Stuart Walsh
title	Myogenic reprogramming of bone marrow derived cells in a W⁴¹Dmd(mdx) deficient mouse model.
title_short	Myogenic reprogramming of bone marrow derived cells in a W⁴¹Dmd(mdx) deficient mouse model.
title_full	Myogenic reprogramming of bone marrow derived cells in a W⁴¹Dmd(mdx) deficient mouse model.
title_fullStr	Myogenic reprogramming of bone marrow derived cells in a W⁴¹Dmd(mdx) deficient mouse model.
title_full_unstemmed	Myogenic reprogramming of bone marrow derived cells in a W⁴¹Dmd(mdx) deficient mouse model.
title_sort	myogenic reprogramming of bone marrow derived cells in a w⁴¹dmd(mdx) deficient mouse model.
publisher	Public Library of Science (PLoS)
publishDate	2011
url	https://doaj.org/article/acfdbed83045419f84768dd6f676bff6
work_keys_str_mv	AT stuartwalsh myogenicreprogrammingofbonemarrowderivedcellsinaw41dmdmdxdeficientmousemodel AT jensnygren myogenicreprogrammingofbonemarrowderivedcellsinaw41dmdmdxdeficientmousemodel AT annicaponten myogenicreprogrammingofbonemarrowderivedcellsinaw41dmdmdxdeficientmousemodel AT stefanjovinge myogenicreprogrammingofbonemarrowderivedcellsinaw41dmdmdxdeficientmousemodel
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Myogenic reprogramming of bone marrow derived cells in a W⁴¹Dmd(mdx) deficient mouse model.

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