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

First Author  Sedehizade F Year  1997
Journal  Muscle Nerve Volume  20
Issue  2 Pages  186-94
PubMed ID  9040657 Mgi Jnum  J:53205
Mgi Id  MGI:1331513 Doi  10.1002/(sici)1097-4598(199702)20:2<186::aid-mus8>3.0.co;2-8
Citation  Sedehizade F, et al. (1997) Expression of nerve-regulated genes in muscles of mouse mutants affected by spinal muscular atrophies and muscular dystrophies. Muscle Nerve 20(2):186-94
abstractText  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.
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