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Terence Partridge

Publications and source records attributed to Terence Partridge.

11 recordsLinked to original sources

Direct isolation of satellite cells for skeletal muscle regeneration.

Muscle satellite cells contribute to muscle regeneration. We have used a Pax3(GFP/+) mouse line to directly isolate (Pax3)(green fluorescent protein)-expressing muscle satellite cells, by flow cytometry from adult skeletal muscles, as a homogeneous population of small, nongranular, Pax7+, CD34+, CD45-, Sca1- cells. The flow cytometry parameters thus established enabled us to isolate satellite cells from wild-type muscles. Such cells, grafted into muscles of mdx nu/nu mice, contributed both to fiber repair and to the muscle satellite cell compartment. Expansion of these cells in culture before engraftment reduced their regenerative capacity.

Animals↗

Delivery of oligodeoxynucleotides into human saphenous veins and the adjunct effect of ultrasound and microbubbles.

Therapy with naked oligodeoxynucleotides (ODNs, molecular weight: 3000 to 7500) provides an elegant means of modulating gene expression without the problems associated with conventional gene therapy, but the relatively low transfer efficiency on intravascular administration is a limitation to clinical application. Ultrasound, which can be potentiated by microbubbles, shows promise as a method of delivering macromolecules such as plasmid DNA and other transgenes into cells. Since uptake of molecules into cells depends on their molecular weight, it might be expected that the delivery of ODNs, which are relatively small, will be facilitated by ultrasound and microbubbles. In the present study, we delivered ODNs into veins using ultrasound and microbubbles. First, we quantified the uptake of fluorescent-labeled ODNs into intact ex vivo human saphenous veins and isolated smooth muscle cells from the veins, evaluating the effect of ultrasound and microbubbles on uptake. Ultrasound potentiated the delivery of ODN in cells, except at high concentrations. When intact veins were studied, we achieved nuclear localization of fluorescent-labeled ODNs in cells. This increased with increasing concentration and incubation time and was not potentiated by ultrasound, even when microbubbles were used. We then applied a therapeutic ODN (antisense to intercellular adhesion molecule 1, ICAM-1) to vein samples and documented a functional inhibition of gene expression in a sequence-specific manner at the protein level with immunohistochemistry and western blot analysis. Again, no significant difference was seen with adjunct ultrasound. These observations suggest high diffusion of ODNs into human saphenous veins in this ex vivo model, indicating potential applications to inhibition of vascular bypass graft occlusion and other vasculopathies. Although microbubble-ultrasound was of value with cells in culture, it was not beneficial with intact veins.

Aged↗

Effects of T-lymphocyte depletion on muscle fibrosis in the mdx mouse.

Duchenne muscular dystrophy was initially described as a myosclerosis because of the conspicuous progression of interstitial fibrosis. Using the mdx mouse homologue, we have shown previously that the accumulation of intramuscular collagen is profoundly influenced by the presence or absence of T lymphocytes. Here we have used thymectomy and antibody depletion to examine the effect of ablating CD4 or CD8 or both subsets of T lymphocytes on skeletal muscle fibrosis in mdx and C57BL10 (wild-type) mice. Depletion of either or both subsets at 4 weeks of age did not influence fibrosis in mdx mice, as determined by measuring hydroxyproline levels and collagen deposition in diaphragm. Additionally, expression of transforming growth factor-beta1, which is implicated in collagen deposition, either decreased (mdx mice) or increased (C57BL/10 mice) after double CD4/8 depletion. Our data suggest that depletion of lymphoid cells may affect the tight regulatory control of transforming growth factor-beta1, with possible pleiotropic effects, and more importantly, that the fibrotic process is self-sustaining from a very early stage.

Animals↗

Systemic delivery of antisense oligoribonucleotide restores dystrophin expression in body-wide skeletal muscles.

Antisense oligonucleotide-mediated alternative splicing has great potential for treatment of Duchenne muscular dystrophy (DMD) caused by mutations within nonessential regions of the dystrophin gene. We have recently shown in the dystrophic mdx mouse that exon 23, bearing a nonsense mutation, can be skipped after intramuscular injection of a specific 2'-O-methyl phosphorothioate antisense oligoribonucleotide (2OMeAO). This skipping created a shortened, but in-frame, transcript that is translated to produce near-normal levels of dystrophin expression. This expression, in turn, led to improved muscle function. However, because DMD affects muscles body-wide, effective treatment requires dystrophin induction ideally in all muscles. Here, we show that systemic delivery of specific 2OMeAOs, together with the triblock copolymer F127, induced dystrophin expression in all skeletal muscles but not in cardiac muscle of the mdx dystrophic mice. The highest dystrophin expression was detected in diaphragm, gastrocnemius, and intercostal muscles. Large numbers of fibers with near-normal level of dystrophin were observed in focal areas. Three injections of 2OMeAOs at weekly intervals enhanced the levels of dystrophin. Dystrophin mRNA lacking the targeted exon 23 remained detectable 2 weeks after injection. No evidence of tissue damage was detected after 2OMeAO and F127 treatment either by serum analysis or histological examination of liver, kidney, lung, and muscles. The simplicity and safety of the antisense protocol provide a realistic prospect for treatment of the majority of DMD mutations. We conclude that a significant therapeutic effect may be achieved by further optimization in dose and regime of administration of antisense oligonucleotide.

Animals↗

Nude mutation influences limb skeletal muscle development.

The nude mouse lacks functional T cells and other skin defects due to the absence of Foxn1 transcription factor. The mdx mouse bred onto a nude background (Foxn1-/-), to generate mdx nu/nu, serves as a valuable model for the use in muscle cell transplantation or gene therapy studies, to avoid a detrimental immune response. However, it has been shown previously that the mdx nu/nu mice have a skeletal muscle specific alteration in collagen production, which in the case of the limb muscles acts at or before the onset of the disease. This led to the conclusion that the nude mutation has an effect on the collagen deposition in skeletal muscles, which may be acting in a T cell independent manner. In an attempt to elucidate the role of the nude mutation on collagen production in skeletal muscles, we have measured collagen levels in several muscles of C57BL/10 nu/nu mice and compared them with mdx nu/nu and Foxn1+/+ control mice. We report here that the limb muscles of the young C57BL/10 nu/nu and mdx nu/nu mice contained a far higher proportion of collagen at an early age than Foxn1+/- control mice but regained normal levels by 12 weeks of age. This was largely attributable to the fact that 3 weeks old nude mice had considerably smaller limb muscles than Foxn1+/- mice which subsequently grew to normal size. We conclude that the nude mutation retards the early growth of development of muscle but not of the matrix tissue in limb muscles.

Animals↗

Reenthronement of the muscle satellite cell.

In this issue of Cell (Sherwood et al., 2004), a quantitative survey confirms that repair of skeletal muscle is overwhelmingly attributable to the endogenous satellite cell population but that experience of a regenerating muscle environment confers some myogenic qualities onto a tiny population of bone marrow-derived cells.

Animals↗

Col1a2 enhancer regulates collagen activity during development and in adult tissue repair.

An enhancer region in the type I collagen alpha 2 chain (pro-Col1a2) promoter has been previously identified approximately -17 kb away from the transcription start site. This upstream region termed the far-upstream-enhancer contains three DNAse I hypersensitive sites and has been shown to be conserved between mouse and human genes. In this study, we used transgenic mice harbouring the complete promotor sequence of the pro-Col1a2 gene up to -17 kb to examine the role of this enhancer in the expression and regulation of the collagen gene during development and in adult tissues pre and post injury. By careful histological mapping of the collagen type I endogenous gene distribution with that of the transgene driven by the mouse far upstream enhancer, we are able to show that in early days of collagen expression, E8.5-9.5, the endogenous gene preceded transgene expression. However, by E11.5 the overall pattern becomes synchronous with a few exceptions. In adult tissue, both endogenous and transgene expression are attenuated and both are reactivated in parallel in various organs by physical injury or fibrogenic cytokine injection. These findings suggest that the enhancer is central to the activation of the collagen type I and that mice harbouring this enhancer/reporter provide a useful model to follow collagen gene transcription activity and for investigating cellular activity in tissue fibrosis.

Animals↗

Relocalization of neuronal nitric oxide synthase (nNOS) as a marker for complete restoration of the dystrophin associated protein complex in skeletal muscle.

A lack of effective treatments for Duchenne muscular dystrophy, a fatal X-linked myopathy, has focused attention on the possibility of gene therapy. The aim of the gene therapy approach is the restoration of the dystrophin associated complex of proteins, one member of which is neuronal nitric oxide synthase, an important enzyme in signal transduction. Transgenic mdx mice and plasmid gene transfer of both human and murine recombinant dystrophins was used to assess whether nNOS could be restored to the sarcolemma following dystrophin gene transfer at a variety of levels of expression. Murine revertant fibres and human patients with different dystrophin deletions were used to assess the relationship between exon deletion and loss of neuronal nitric oxide synthase localization to the sarcolemma. We demonstrate that the domain encoded by exons 45-48 is required for localization of neuronal nitric oxide synthase to the sarcolemma. On the basis of these observations we suggest that neuronal nitric oxide synthase is a useful marker for complete restoration of the dystrophin associated complex and should be used as one of the criteria for selecting the recombinant molecule to be used for gene therapy in Duchenne muscular dystrophy.

Adult↗

Muscle stem cells.

Since its discovery four decades ago, the satellite cell of skeletal muscle has been implicated as the major source of myogenic cells involved in growth and repair of muscle fibres. This review not only looks at the role of the satellite cell in these processes but discusses how cells derived from other sources and tissues have recently been implicated in muscle formation and regeneration. Muscle itself also yields cells that contribute to other cell lineages although it is currently debated as to whether these cells originate within muscle or have migrated there from other tissues. The reality of using cells from muscle or other tissues to repair diseased muscle fibres is also addressed.

Bone Marrow Cells↗

Identification of the key regions within the mouse pro-alpha 2(I) collagen gene far-upstream enhancer.

Studies using transgenic mice have shown that the mouse pro-alpha2(I) collagen gene contains a far-upstream enhancer, which directs expression in the majority of collagen I-producing cells during development and in response to tissue injury. In this study, we have investigated the minimal functional region required for the enhancer effect and studied the role of the three hypersensitive sites (HS3-HS5) that overlap this region. The results of deletion experiments indicate that the minimal functional unit of this enhancer is a 1.5-kb region between -17.0 and -15.45 kb from the transcription start site. This region includes the core sequences of HS3 and HS4 but not HS5. The HS4 sequences are essential for the functional integrity of the enhancer, whereas HS3 represents tissue-specific elements that direct expression in mesenchymal cells of internal tissues and body wall muscles. The HS3 region appears to bind a complex of transcription factors illustrated by large regions of protected sequences. A 400-bp sequence located between -17.0 and -16.6 is also essential for the enhancer because its deletion results in increased susceptibility to the chromatin environment.

Animals↗