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

Abstract

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

Normal growth and regenerating ability of myoblasts from unaffected muscles of facioscapulohumeral muscular dystrophy patients

“…[55][56][57][58] In the present study, human FSHD myoblasts were injected into immunodeficient Rag mice, a validated mouse model for the analysis of muscle regeneration in the context of xenogenic MT. The implantation results we obtained were close to that reported previously [47][48][49] and confirmed that myoblasts prepared from unaffected FSHD muscles, even used at the 6th and 7th passages, take part into in vivo muscle regeneration. The overall number of dystrophin-positive fibers was inferior to that reported previously in immunocompetent models of muscular dystrophies in the context of syngenic or allogenic MT.…”

“…In the present study, the evaluation of transplantation outcome has been performed 1 month after transplantation, a time course that is of general use in human and animal MT assays. 9,16,17,20,31,47,49 This model was not regarded helpful to evaluate the longterm persistence of muscle fibers harboring the human mutated genes. Indeed, a negative selection of muscle fibers expressing the mutated gene could occur with time in the healthy environment of mouse muscle fibers, and this selection would not be representative of the situation presented in FSHD.…”

“…At 1 month after MT, the expression of human proteins validated the implantation of human cells into mouse muscle fibers. 16,[47][48][49] Human dystrophin was observed in all cell-injected muscles, and was absent from muscles injected with a saline solution (Table 3, Figure 5). No significant differences were observed between muscles receiving FSHD or control myoblasts (P40.8).…”

“…The mice were killed 4 weeks after the last series of cell injections, which is a delay compatible with complete mouse muscle regeneration and the expression of donor markers. 16,[47][48][49] The muscles were snap frozen in nitrogen-cooled isopentan and serially sectioned (8 mm) using a cryostat. The participation of human cells in muscle formation was detected by the specific expression of human dystrophin beneath the basement membrane of muscle fibers.…”

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

“…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%).…”