SCINT II: An improved program for the statistical analysis and plotting of variably quenched dual-labeled liquid scintillation samples

Duchenne muscular dystrophy (DMD), the most common and severe form of the muscular dystrophies, is an X-linked inborn error of metabolism with multiple tissue involvement. Although the major pathological changes are observed in skeletal muscle, abnormalities have also been detected in the heart, nervous system, red blood cells, lymphocytes and cultured skin fibroblasts. For many reasons, such as readily available tissue material, fewer secondary changes and the potential for prenatal diagnosis, cultured skin fibroblasts should be the tissue of choice to search for the primary defect. Several abnormalities have been reported in DMD fibroblasts, suggesting that the genetic abnormality is expressed in these cells. To search for potentially mutant protein(s) we have compared the protein composition of normal and DMD fibroblasts by two-dimensional gel electrophoresis and have now found one protein spot consistently missing in DMD cells. The nature of this protein and its relation to the DMD gene are unknown.

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Biochemical Aspects of Muscle Necrosis in Hamster Dystrophy

Wrogemann¹,

Hayward²,

Blanchaer³

1979

Ann NY Acad Sci

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SCINT II: An improved program for the statistical analysis and plotting of variably quenched dual-labeled liquid scintillation samples

Cited by 5 publications

References 4 publications

Proline incorporation by cultured skin fibroblasts from patients with Duchenne muscular dystrophy

Proline incorporation by cultured skin fibroblasts from patients with Duchenne muscular dystrophy

Analysis of fibroblast proteins from patients with Duchenne muscular dystrophy by two-dimensional gel electrophoresis

Biochemical Aspects of Muscle Necrosis in Hamster Dystrophy

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