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Neurochemical Journal

, Volume 12, Issue 4, pp 305–310 | Cite as

The Structural and Functional Characteristics of the Motor End Plates of Dysferlin-Deficient Mice

  • V. V. Kravtsova
  • N. A. Timonina
  • G. F. Zakir’yanova
  • A. V. Sokolova
  • V. M. Mikhailov
  • A. L. Zefirov
  • I. I. KrivoiEmail author
Experimental Articles
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Abstract

The molecular mechanisms that underlie neuromuscular junction plasticity are complex and remain to be fully elucidated. Experimental models of various forms of impaired motor activity may be promising for their study. The dysferlin protein plays a key role in the multimolecular complex, which maintains sarcolemma integrity and the functioning of skeletal muscle cells. We studied the structural and functional characteristics of the diaphragm muscle motor end plates of dysferlin-deficient Bla/J mice (a model of dysferlinopathy), dystrophin-deficient mdx mice (a model of Duchenne muscular dystrophy), and control C57Bl/6 mice. Increased end plate fragmentation and a decrease in the area of individual fragments were observed in mdx mice and absent in Bla/J mice, which indicates a difference in these models of myodystrophy from these characteristics. However, end plates of both mice lines were characterized by a decrease in the density of distribution of nicotinic acetylcholine receptors, as well as by membrane depolarization, presumably, due to altered functional interaction between the α2 isoform of Na,K-ATPase and the nicotinic acetylcholine receptors.

Keywords

skeletal muscle motor endplate nicotinic acetylcholine receptors dysferlin dysferlinopathy 

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Copyright information

© Pleiades Publishing, Ltd. 2018

Authors and Affiliations

  • V. V. Kravtsova
    • 1
  • N. A. Timonina
    • 1
  • G. F. Zakir’yanova
    • 2
  • A. V. Sokolova
    • 3
  • V. M. Mikhailov
    • 3
  • A. L. Zefirov
    • 4
  • I. I. Krivoi
    • 1
    • 5
    Email author
  1. 1.St. Petersburg State UniversitySt. PetersburgRussia
  2. 2.Kazan Institute of Biochemistry and Biophysics, Federal Research Center Kazan Scientific CenterRussian Academy of SciencesKazanRussia
  3. 3.Institute of CytologyRussian Academy of SciencesSt. PetersburgRussia
  4. 4.Kazan State Medical UniversityKazanRussia
  5. 5.St. PetersburgRussia

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