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Abstract

Human embryonic myogenic precursors were transplanted into muscles of mdx mice with hereditary dystrophin-deficient muscular dystrophy. Transplantation induced the synthesis of human dystrophin. The number of dystrophin-positive fibers progressively decreased, however, some of them were preserved even 5 months after transplantation. Our results indicate that xenogeneic transplantation of embryonic myogenic precursors compensates the genetic defect in dystrophin-deficient mice.

Hereditary muscular dystrophy in MDX mice as a homologous model for introduction of cell technologies in the treatment of progressive muscular dystrophies in humans

“…The results obtained in our studies on mdx mice [3] confirm good prospects of this trend of investigations.…”

“…Patients with PMD often develop dilatation cardiomyopathy associated with arrhythmia [3], and biopsy specimens of the myocardium contain no dystrophin [6]. The same changes were detected in mdx mice [13].…”

“…The presence of the AAT triplet (stop-codon during transcription of full-length dystrophin mRNA) was regarded as a specific characteristic of mdx mouse genome [3].…”

“…Dystrophin was detected by immunohistochemical analysis of cryostat sections of skeletal muscles [3]. Dys1 and Dys2 antibodies (Novocastra) to epitopes of the dystrophin core and C-terminal fragments were used.…”

Cardiotropic effect of extracardiac transplantation of embryonic human myoblasts to mice with bradycardia: Various effects of cell material

“…Human embryonic myoblasts injected into skeletal muscles of mdx mice with hereditary muscular dystrophy partially restored the expression of dystrophin protein in myofibrils [3]. Studies of the PMD cell therapy are mainly focused on reparative processes in skeletal muscles, while the involved heart [1,4] is neglected, though the effects of injected stem cells can be observed also in the heart.…”