Experimental regeneration in canine muscular dystrophy-1. Immunocytochemical evaluation of dystrophin and β-spectrin expression

C. A. Sewry, L. A. Wilson, L. Dux, V. Dubowitz, B. J. Cooper

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12 Citations (Scopus)

Abstract

The expression of dystrophin and β-spectrin was examined from 1 to 56 days in regenerating muscle fibres in normal and dystrophic dogs, following necrosis induced by the venom of Notechis scutatis. Normal and dystrophic dog muscle regenerated at an equal rate and new myotubes were present in both at the periphery of necrotic fibres by 3 days. In normal dogs dystrophin was detected in the sarcoplasm of the regenerating fibres by 3 days and was localized to the plasma membrane by 4 days. The localization of dystrophin is independent of β-spectrin and was detected before β-spectrin, which was not observed until 5-6 days. Normal peripheral labelling of both was restored by 14 days in normal dogs. Normal β-spectrin labelling of regenerating dystrophic fibres was also restored by 14 days and is not dependent on the presence of dystrophin in dystrophic dogs. A proportion of regenerating fibres in normal and dystrophic dogs showed weak immunolabelling of β-spectrin prior to 14 days. This is a feature of immature muscle fibres. Antibodies to different domains of dystrophin bound to the periphery and sarcoplasm of regenerating fibres in dystrophic dogs, particularly during the first 7 days of regeneration, but the fluoresence was less intense than in normal dogs. Weak labelling with antibodies corresponding to the C-terminus of the rod domain of dystrophin persisted on dystrophic regenerating fibres up to 21 days. This may relate to developmental isoforms of dystrophin.

Original languageEnglish
Pages (from-to)331-342
Number of pages12
JournalNeuromuscular Disorders
Volume2
Issue number5-6
DOIs
Publication statusPublished - 1992

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Keywords

  • Dystrophin
  • dystrophic dog
  • muscular dystrophy
  • regeneration
  • β-spectrin

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Clinical Neurology
  • Genetics(clinical)

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