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Abstract
Concentrations of Br, Ca, Cl, K and Na in whole blood of dystrophin-deficient mouse [the Dmdmdx line] were determined using NAA, resulting in reference values that are relevant for clinical blood investigation. The comparison with human being whole blood values was also performed in order to establish possible indexes and similarities among the experimental and clinical applications.
22 228 234 Chamberlain, S. J., Metzger, J., Reyes, M., Townsend, D., Faulkner, A. J. (2007) Dystrophin-deficient mdx mice display a reduced life
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Hoffman, E. P., Brown, R. H., Kunkel, L. M.: Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell, 1987, 51 , 917–928. Kunkel L. M. Dystrophin: the
Two related European Grey wolves ( Canis lupus ) with the history of muscle stiffness beginning at 2 weeks of age were examined in this study. Muscle tone and muscle mass were increased in both animals. Muscle stiffness was worsened by stress so that the animals fell into lateral recumbency. Blood chemistry revealed mildly increased serum creatine kinase activity. Abnormal potentials typical of myotonic discharges were recorded by electromyography. Cataract, first-degree atrioventricular (AV) block and inhomogeneous myocardial texture by ultrasound suggested extramuscular involvement. Myopathology demonstrated dystrophic signs in the muscle biopsy specimen. The presumptive diagnosis based on the in vivo findings was myotonic dystrophy. Immunochemistry of the striated muscles revealed focal absence of dystrophin 1 and beta-dystroglycan in both cases. Cardiac and ophthalmologic involvement suggested a disorder very similar to a human form of myotonic dystrophy. This is the first description of myotonic dystrophy in wolves.
168 Thomas, G.D., Sander, M., Lau, K.S., Huang, P.L., Stull, J.T., Victor, R.G.: Impaired metabolic modulation of alpha-adrenergic vasoconstriction in dystrophin-deficient skeletal muscle
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