Transplantation of myoblasts from a transgenic mouse overexpressing dystrophin produced only a relatively small increase of dystrophin-positive membrane

Kinoshita, Ikuo; Vilquin, Jean‐Thomas; Asselin, Isabelle; Chamberlain, Jeffrey S.; Tremblay, Jacques P.

doi:10.1002/(sici)1097-4598(199801)21:1<91::aid-mus12>3.0.co;2-3

Cited by 51 publications

(34 citation statements)

References 41 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Our studies confirm some previous findings obtained in other models, ie over 1500 m for ␤-gal 60,61 and about 300 m for dystrophin. 60,62 Along the same line, we show here that the domain of expression of the laminin-2 molecule is approximately 500 m. These results underline one present limitation of direct gene transfer into skeletal muscles, which is the restricted segmental diffusion of the transgenic proteins inside the transduced fibers. This may be considered an advantage in situations where the local delivery of a protein is required.…”

Section: Figure 7 Lack Of Persistence Of Transduced Fibers In An Acutsupporting

confidence: 80%

See 1 more Smart Citation

Electrotransfer of naked DNA in the skeletal muscles of animal models of muscular dystrophies

Vilquin

Kennel²,

Paturneau-Jouas

et al. 2001

Gene Ther

View full text Add to dashboard Cite

The electrotransfer of naked DNA has recently been adapted to the transduction of skeletal muscle fibers. We investigated the short-and long-term efficacy of this methodology in wild-type animals and in mouse models of congenital muscular dystrophy (dy/dy, dy 2J /dy 2J ), or Duchenne muscular dystrophy (mdx/mdx). Using a reporter construct, the short-term efficacy of fiber transduction reached 40% and was similar in wild-type, dy/dy and dy 2J /dy 2J animals, indicating that ongoing muscle fibrosis was not a major obstacle to the electrotransfer-mediated gene transfer. Although the complete rejection of transduced fibers was observed within 3 weeks in the absence of immunosuppression, the persistency was prolonged over 10 weeks when transient or continuous immunosuppressive regimens were used. Using

show abstract

Section: Figure 7 Lack Of Persistence Of Transduced Fibers In An Acutsupporting

confidence: 80%

“…Such differences were already observed in cell transplantation experiments. 60,61 Third, the plasmid constructions and preparations differ. The ␤-gal plasmid has a pCOR backbone and, due to its size and construction, may be present in a supercoiled form at a very high percentage (Ͼ99%).…”

Section: Figure 7 Lack Of Persistence Of Transduced Fibers In An Acutmentioning

confidence: 99%

Electrotransfer of naked DNA in the skeletal muscles of animal models of muscular dystrophies

Vilquin

Kennel²,

Paturneau-Jouas

et al. 2001

Gene Ther

View full text Add to dashboard Cite

show abstract

“…Laminin-2 and dystrophin, however, do present intrinsic differences such as molecular weight and subcellular localization, which may account for the differential detection of the proteins observed in some cases, as illustrated in a previous study in another model. 34 Our present results indicate mainly that the distance of migration would be limited, since most of the fibers displaying the presence of laminin-2 were restricted to the injection sites.…”

Section: Discussionmentioning

confidence: 56%

Myoblast transplantations lead to the expression of the laminin α2 chain in normal and dystrophic (dy/dy) mouse muscles

et al. 1999

View full text Add to dashboard Cite

Laminin-2 is part of the basement membrane of the skelmouse myoblast cell line in dy/dy muscles allows the etal muscle fibers. The laminin ␣2 chain is absent or drastiexpression of the murine laminin ␣2 chain; and (3) allocally reduced in a subgroup of congenital muscular dystransplantation of the D7 dystrophic dy/dy cell line allows trophy patients, and in the severely affected dystrophic the formation of new and hybrid muscle fibers in dy/dy dy/dy mouse. We previously reported that heterogenous muscle in the absence of laminin ␣2 chain expression. We primary mouse muscle cell cultures conferred laminin ␣2 conclude that normal myoblasts are able to restore the chain expression in dy/dy mice muscles upon cell transexpression of an extracellular skeletal muscle protein and plantation. In the present study we investigated whether that the absence of laminin-2 does not prevent transpure myoblast cell lines were able to confer laminin ␣2 planted muscle cells from participating in the formation of chain expression in vivo. We observed that: (1) xenomyofibers. Myoblasts are, therefore, attractive tools for transplantation of non-immortalized human myoblast in further exploration of gene complementation strategies in SCID mouse muscles allows human laminin ␣2 chain the animal models of congenital muscular dystrophy. expression; (2) allotransplantation of the permanent G8

show abstract

“…Indeed, our group has shown that, when a myoblast fuses to a preformed fiber and expresses both ␤-Gal and dystrophin, the former protein diffuses further from the point of its translation due to its smaller size and cytosolic localization, thus rendering it detectable on more serial sections of a muscle. 30 For technical reasons, only one cryosection per muscle has been counted. It is therefore possible that a given ␤-Gal + /Dys − fiber in a section is ␤-Gal + /Dys + in another section that is closer to where the myoblast initially fused.…”

Section: Discussionmentioning

confidence: 99%

Expression of human dystrophin following the transplantation of genetically modified mdx myoblasts

Gagnon

Karpati

et al. 1998

Gene Ther

View full text Add to dashboard Cite

Transplantation of genetically modified autologous myoof SCID mice. Ten and 24 days after transplantation, blasts has been proposed as a possible solution to avoid numerous muscle fibers expressing both human dystrophin long-term use of immunosuppressive drugs. To determine and ␤-Gal were detected throughout the mouse muscles the conditions to be used in this kind of approach for possby immunohistochemistry using an antibody specific for ible treatment of dystrophin deficiency, mdx myoblasts human dystrophin. The presence of the human mini-dystrowere infected at different multiplicities of infection (MOI of phin mRNA was also detected by RT-PCR. These results 0.01-1000) with an adenoviral vector containing a CMV demonstrate that three essential conditions in autologous promoter/enhancer driven 6.3 kb human dystrophin cDNA myoblast transplantation can be achieved: (1) in vivo sur-(minigene) and tested in vitro for transgene expression. In vival of at least some of the transduced myoblasts; (2) these cultures, dystrophin mRNA was found to be proefficient fusion of these cells with the host muscle fibers; portionate with increasing MOI. Primary myoblast cultures and (3) the high expression of the dystrophin transgene in derived from transgenic mdx mice expressing ␤-Gal under situ. Furthermore, this article provides a novel RT-PCRa muscle-specific promoter and showing high expression based technique to quantify the human dystrophin miniof the human mini-dystrophin transgene introduced by the gene expression in vitro and in vivo. adenoviral vector were grafted into anterior tibialis muscles

show abstract

Transplantation of myoblasts from a transgenic mouse overexpressing dystrophin produced only a relatively small increase of dystrophin-positive membrane

Cited by 51 publications

References 41 publications

Electrotransfer of naked DNA in the skeletal muscles of animal models of muscular dystrophies

Electrotransfer of naked DNA in the skeletal muscles of animal models of muscular dystrophies

Myoblast transplantations lead to the expression of the laminin α2 chain in normal and dystrophic (dy/dy) mouse muscles

Expression of human dystrophin following the transplantation of genetically modified mdx myoblasts

Contact Info

Product

Resources

About