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Gene Review

pma  -  peroneal muscular atrophy

Mus musculus

 
 
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Disease relevance of pma

 

High impact information on pma

 

Biological context of pma

  • Linkage analyses using backcross progeny revealed a significant linkage between the pma gene and three microsatellite markers, D5Mit263 at 73 cM, D5Mit141 and D5Mit97 at 74 cM on Chr 5 [5].
  • The effect of TTX shows that aneural pma primary myotubes discharge spontaneous myogenic action potentials, while mdg muscles may receive greater than normal electrical activation due to their hyperinnervation, explaining the presence and numbers of secondary myotubes in the mutant mouse muscles [3].
  • Regulation of myogenesis in paralyzed muscles in the mouse mutants peroneal muscular atrophy and muscular dysgenesis [3].
 

Anatomical context of pma

  • Secondary myotubes appeared at their normal time and position within the muscle, initially in normal numbers, so that the ratio of secondary to primary myotubes initially was greater in pma than in CF1 control strain mice [4].
  • Therefore, the absence of the common peroneal nerve in the pma mouse is thought to result from the misdirection of these nerve bundles into other peripheral nerves [1].

References

  1. Hereditary peroneal muscular atrophy in the mouse: an experimental model for congenital contractures (arthrogryposis). Nonaka, I., Kikuchi, A., Suzuki, T., Esaki, K. Exp. Neurol. (1986) [Pubmed]
  2. Targeting of the EphA4 tyrosine kinase receptor affects dorsal/ventral pathfinding of limb motor axons. Helmbacher, F., Schneider-Maunoury, S., Topilko, P., Tiret, L., Charnay, P. Development (2000) [Pubmed]
  3. Regulation of myogenesis in paralyzed muscles in the mouse mutants peroneal muscular atrophy and muscular dysgenesis. Ashby, P.R., Pinçon-Raymond, M., Harris, A.J. Dev. Biol. (1993) [Pubmed]
  4. Formation of primary and secondary myotubes in aneural muscles in the mouse mutant peroneal muscular atrophy. Ashby, P.R., Wilson, S.J., Harris, A.J. Dev. Biol. (1993) [Pubmed]
  5. Chromosomal mapping of the peroneal muscular atrophy (pma) gene in the mouse. Katoh, H., Watanabe, Y., Ebukuro, M., Muguruma, K., Takabayashi, S., Shiroishi, T. Exp. Anim. (2003) [Pubmed]
 
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