A histological study of muscle from the bar harbor strain of dystrophic mice

Pearce, G. W.; Walton, John N.

doi:10.1002/path.1700860105

Cited by 51 publications

(18 citation statements)

References 8 publications

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“…Morphological differences between the dy mutant and Duchenne dystrophy were apparent to early investigators (23) and somewhat similar discrepancies were found between mdx mutants and human dystrophy. Whether these variations indicate a different etiology or a species response is unknown.…”

Section: Discussionsupporting

confidence: 55%

X chromosome-linked muscular dystrophy (mdx) in the mouse.

Bulfield¹,

Siller²,

Wight³

et al. 1984

Proc. Natl. Acad. Sci. U.S.A.

1,522

1,041

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An X chromosome-linked mouse mutant (gene symbol, mdx) has been found that has elevated plasma levels of muscle creatine kinase and pyruvate kinase and exhibits histological lesions characteristic of muscular dystrophy. The mutants show mild clinical symptoms and are viable and fertile. Linkage analysis with four X chromosome loci indicates that mdx maps in the Hq Bpa region of the mouse X chromosome. This gives a gene order of mdx-Tfm-Pgk-1-Ags, the same as for the equivalent genes on the human X chromosome.There are two major forms of X chromosome-linked muscular dystrophy in man: Duchenne (McKusick number, 31020) and Becker (McKusick number, 31010) (1-3); recent linkage analyses with restriction fragment-length polymorphisms suggests that they could be allelic (4, 5). For neither of these syndromes is the primary lesion or a homologous animal model known. Several mouse mutants exhibit myopathies (6, 7); the most investigated of these are the autosomal dy and dy2 mutants (6). There is, however, controversy over whether the dy mutants are myogenic or neurogenic in origin (8-10) and, therefore, their suitability as animal models of Duchenne/Becker muscular dystrophy (11).Therefore, it is of considerable interest that a spontaneous mutation (mdx) arose in our inbred C57BL/10 colony of mice that is X chromosome-linked and produces viable homozygous animals that have high serum levels of muscle enzymes and exhibit histological lesions similar to human muscular dystrophy.MATERIALS AND METHODS Animals. C57BL/lOScSn inbred mice were originally obtained from M. Festing (MRC Laboratory Animals Centre, Carshalton, Surrey, U.K.) and further inbred for five generations prior to the spontaneous appearance of the X chromosome-linked muscular dystrophy (mdx) mutation. Segregation analysis was performed on animals within this stock. The position of mdx on the X chromosome was located by crossing to four mutants: Mottled-blotchy (Moblo), Tabby (Ta), sparse-fur (spf) and Hypophosphataemia (Hyp) (all the gift of M. Lyon, MRC Radiobiology Unit, Harwell, Oxon, U.K.).Enzyme Assays. Pyruvate kinase (PK; EC 2.7.1.40) activity was determined by a semiautomated enzyme assay (12) and expressed as urmol/min per ml of whole blood ± SEM. In some experiments the blood was fractionated into plasma and erythrocytes after withdrawal by heart puncture into heparinized tubes. The blood was centrifuged at 1,000 x gavg for 15 min, and the plasma was withdrawn; the leukocyte and interface layers were discarded, and the erythrocytes were resuspended twice in 0.15 M NaCl and recentrifuged and then were lysed in 0.05 M Tris, 7.4/1 mM EDTA/1 mM dithiothreitol/0.1% Triton X-100. Cellulose acetate electrophoresis and staining of creatine kinase (EC 2.7.3.2) was performed according to the protocol provided by Helena Laboratories (Beaumont, TX).Histology. All mice were examined clinically before being killed, and as many muscles as possible were surveyed. Ether-killed mice were skinned, eviscerated, fixed in 10% Formal/saline, halved in the median...

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Section: Discussionsupporting

confidence: 55%

X chromosome-linked muscular dystrophy (mdx) in the mouse.

Bulfield¹,

Siller²,

Wight³

et al. 1984

Proc. Natl. Acad. Sci. U.S.A.

1,522

1,041

View full text Add to dashboard Cite

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“…There was no arrangement of atrophied fibres in groups or fascicles characteristic of partial motor denervation due to structural loss of motor nerve fibres as in human motor system diseases or peripheral neuropathies (Adams, Denny-Brown, and Pearson, 1962) and in the wobbler mouse. In none of the medmice examined histologically was necrosis of muscle fibres seen and the appearances were vtry unlike the changes which have been described in hereditary dystrophia muscularis in the mouse (Michelson et al, 1955;West and Murphy, 1960;Pearce and Walton, 1963;Banker, 1967) or in other primary myopathic diseases (Adams et al, 1962).…”

Section: Discussionmentioning

confidence: 75%

Hereditary motor end-plate disease in the mouse: light and electron microscopic studies.

Duchen¹

1970

Journal of Neurology, Neurosurgery & Psychiatry

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“…The pathology of murine dystrophy has been extensively reported (West and Murphy, 1960;Pearce and Walton, 1963) and there is general agreement on the main features of dystrophic muscle (fibre atrophy and hypertrophy, central nucleation, proliferation of connective tissue, necrosis, and phagocytosis). It is not surprising that under these conditions the twitch kinetics of the muscles are changed.…”

Section: Each Increment In Tension Is Due To Thementioning

confidence: 90%