Expression of dystrophin in the mouse myenteric neurones

Neurosci Lett. 2001 Mar 9;300(2):120-4. doi: 10.1016/s0304-3940(01)01555-5.

Abstract

Dystrophin, a membrane-associated protein, plays relevant roles in cell functions. Its lack or trunkated expression results in Duchenne muscular dystrophy (DMD), a pathology associated with alterations in gastrointestinal motility considered to be neural in origin. No data are available on the presence of dystrophin in myenteric neurones. We labelled mouse myenteric neurones with DYS1-, DYS2-, DYS3-antibodies; staining was located on the perikarya and processes, with no differences in distribution or intensity among the antibodies; the western immunoblot analysis indicated that myenteric neurones express several dystrophin isoforms; anti-dystrophins/anti-neuronal specific enolase double-labeling confirmed that all neurones express dystrophin. Dystrophin in myenteric neurones might play a role in cytoskeletal organization, axonal transport and signal pathways; its lack might cause the intestinal motor abnormalities reported in DMD patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Digestive System / cytology
  • Digestive System / innervation*
  • Digestive System / metabolism
  • Dystrophin / metabolism*
  • Gastrointestinal Diseases / etiology
  • Gastrointestinal Diseases / metabolism
  • Gastrointestinal Diseases / physiopathology
  • Gastrointestinal Motility / physiology*
  • Immunohistochemistry
  • Male
  • Mice
  • Muscular Dystrophy, Duchenne / complications
  • Muscular Dystrophy, Duchenne / metabolism
  • Muscular Dystrophy, Duchenne / physiopathology
  • Myenteric Plexus / cytology
  • Myenteric Plexus / metabolism*
  • Neurons / metabolism*
  • Phosphopyruvate Hydratase / metabolism
  • Protein Isoforms / metabolism
  • Protein Structure, Tertiary / physiology

Substances

  • Dystrophin
  • Protein Isoforms
  • Phosphopyruvate Hydratase

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