Purine Metabolism in Fibroblasts of Patients with Duchenne&amp;rsquo;s Muscular Dystrophy

Willers, I.; Singh, Shailendra; Goedde, H. W.

doi:10.1159/000153299

Cited by 3 publications

References 8 publications

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Tissue culture studies of muscle disorders: Part 1. Techniques, cell growth, morphology, cell surface

Witkowski¹

1986

Muscle and Nerve

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Tissue culture has been used extensively in studies of human inherited disorders, and its application in the field of the neuromuscular disorders has increased rapidly in recent years. This review, covering the period 1977 to 1984, deals with tissue culture studies of both human and animal muscle disorders, although Duchenne muscular dystrophy (DMD) figures prominently because of the overwhelming interest in that disorder. The review is in two parts. In the first part, I discuss technical innovations in the field, the morphology and growth of cells, and a variety of studies related to the cell surface. Important findings in relation to DMD include reports of abnormal growth rates and reduced lifespan of DMD cells, hypersensitivity to DNA-damaging agents, abnormal cell-to-cell and cell-to-substratum adhesion, and a more "fluid" cell membrane. However, these findings are controversial or have so far been reported only by single laboratories.

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Tissue culture studies of muscle disorders: Part 1. Techniques, cell growth, morphology, cell surface

Witkowski¹

1986

Muscle and Nerve

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Tissue culture studies of muscle disorders: Part 2. Biochemical studies, nerve‐muscle culture, metabolic myopathies, and animal models

Witkowski¹

1986

Muscle and Nerve

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This review continues with studies of protein, lipid, and purine metabolism of Duchenne muscular dystrophy (DMD) cells in vitro and of muscle cells in combined culture with nerve cells. In vitro studies of human metabolic myopathies are tabulated. Results using the hamster, chicken, and mouse (dy25, dy, mdg, and mdx) myopathies are discussed. Interesting findings include suggestions of altered collagen synthesis by DMD cells. Analysis of cell proteins by two-dimensional gel electrophoresis and the use of combined nerve-muscle cultures remain important areas of development. It is disappointing that so few attempts have been made to repeat significant findings in this field, and when a number of laboratories have examined the same phenomenon, the results are often contradictory. It remains to be shown how these various abnormalities found in cells in vitro are related to each other and to those pathologic features of diseased muscle observed in vivo.

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The Study of Adenosine Metabolism in Isolated Cells and Tissues

Henderson

1985

Methods Used in Adenosine Research

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Purine Metabolism in Fibroblasts of Patients with Duchenne’s Muscular Dystrophy

Cited by 3 publications

References 8 publications

Tissue culture studies of muscle disorders: Part 1. Techniques, cell growth, morphology, cell surface

Tissue culture studies of muscle disorders: Part 1. Techniques, cell growth, morphology, cell surface

Tissue culture studies of muscle disorders: Part 2. Biochemical studies, nerve‐muscle culture, metabolic myopathies, and animal models

The Study of Adenosine Metabolism in Isolated Cells and Tissues

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