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Myogenic Reprogramming of Bone Marrow Derived Cells in a WDmd Deficient Mouse Model.

Walsh, Stuart; Nygren, Jens LU ; Pontén, Annica LU and Jovinge, Stefan LU (2011) In PLoS One 6(11).
Abstract
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... (More)
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 (W(41)) mdx mice. This model allows BMDC reconstitution in the absence of irradiation induced myeloablation. We provide the first report of BMDC fusion in a W(41)/Dmd(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 W(41)Dmd(mdx) mice would be similar to frequencies observed in infarcted mice [1].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. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
PLoS One
volume
6
issue
11
publisher
Public Library of Science
external identifiers
  • WOS:000298163400007
  • PMID:22140444
  • Scopus:82155181957
ISSN
1932-6203
DOI
10.1371/journal.pone.0027500
language
English
LU publication?
yes
id
8f4e4ecc-e94e-40b6-aa56-cd1ecbf0a448 (old id 2274577)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/22140444?dopt=Abstract
date added to LUP
2012-01-03 12:13:25
date last changed
2016-10-13 04:27:36
@misc{8f4e4ecc-e94e-40b6-aa56-cd1ecbf0a448,
  abstract     = {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 (W(41)) mdx mice. This model allows BMDC reconstitution in the absence of irradiation induced myeloablation. We provide the first report of BMDC fusion in a W(41)/Dmd(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 W(41)Dmd(mdx) mice would be similar to frequencies observed in infarcted mice [1].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.},
  author       = {Walsh, Stuart and Nygren, Jens and Pontén, Annica and Jovinge, Stefan},
  issn         = {1932-6203},
  language     = {eng},
  number       = {11},
  publisher    = {ARRAY(0x88659c0)},
  series       = {PLoS One},
  title        = {Myogenic Reprogramming of Bone Marrow Derived Cells in a WDmd Deficient Mouse Model.},
  url          = {http://dx.doi.org/10.1371/journal.pone.0027500},
  volume       = {6},
  year         = {2011},
}