Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies

Muscle Nerve. 1997 Feb;20(2):186-94. doi: 10.1002/(sici)1097-4598(199702)20:2<186::aid-mus8>3.0.co;2-8.

Abstract

The expression of the genes for the alpha-subunit of AChR (AChR alpha), for the myogenic factors myogenin and MyoD, for the calcium-binding protein parvalbumin (PV), and for the muscular chloride channel CIC-1 was studied in the three mouse spinal muscular atrophies (SMAs). These were the mutants "wobbler" (WR), "muscle deficient" (MDF) and "progressive motor neuronopathy" (PMN). Murine myopathies "muscular dystrophy with myositis" (MDM) and "X-linked muscular dystrophy" (MDX) were used as controls. AChR alpha and myogenin mRNA levels were strongly elevated in muscles affected by SMAs (reflecting denervation), whereas only myogenin mRNA was moderately elevated in MDX and MDM muscles, probably due to fiber regeneration. As in denervated muscle, CIC-1 and PV mRNA levels were lowered in SMAs. No changes were seen in muscles of up to 222-day-old symptomless ciliary neurotrophic factor (CNTF) knockout mice. The patterns of gene expression were characteristic for the type of muscle disease, indicating their possible usefulness for clinical diagnosis.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Blotting, Northern
  • Disease Models, Animal
  • Female
  • Gene Expression Regulation / physiology
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Inbred mdx
  • Muscle, Skeletal / chemistry
  • Muscle, Skeletal / innervation
  • Muscular Atrophy, Spinal / genetics*
  • Muscular Atrophy, Spinal / physiopathology*
  • Muscular Dystrophy, Animal / genetics*
  • Muscular Dystrophy, Animal / physiopathology
  • MyoD Protein / genetics
  • Myogenin / genetics
  • Peripheral Nerves / physiology*
  • RNA, Messenger / analysis

Substances

  • MyoD Protein
  • Myog protein, mouse
  • Myogenin
  • RNA, Messenger