title: Interstitial cells of Cajal, enteric neurons, and smooth muscle and myoid cells of the murine gastrointestinal tract express full-length dystrophin.
abstract: A gene located on the X chromosome is responsible for the transcription of several mRNA and related dystrophin isoforms. Lack or truncated expression of the 427-kDa, full-length isoform in skeletal muscle results in Duchenne muscular dystrophy (DMD). Patients with DMD, as well as mdx mice, a mutant strain also lacking this isoform, show gastrointestinal dismotilities. The present aim was to identify the cell types that express full-length dystrophin in the gastrointestinal tract. An immunohistochemical study was performed using an antibody specific for this isoform, and double labelings were made for interstitial cells of Cajal (ICC) identification and to verify whether all neurons express full-length dystrophin. Three different fixation procedures were used. The results showed that ICC, enteric neurons, and smooth muscle and myoid cells expressed full-length dystrophin. In ICC and neurons, dystrophin-immunoreactive patches were irregularly distributed at the cell contour and within the cytoplasm. In smooth muscle and myoid cells, regularly spaced dystrophin-immunoreactive bars were located along the cell contour. Labeling intensity varied according to fixation procedure. The different subcellular distributions of dystrophin immunoreactivity might reflect diverse roles played by full-length isoforms in each cell type. Dystrophin loss in cells involved in gastrointestinal motility might explain the gastrointestinal symptomatology affecting DMD patients and mdx mice.
authors.affiliation: Department of Human Anatomy, Histology and Forensic Medicine, Section of Histology E Allara, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy.
authors.forename: Maria-Giuliana
authors.initials: MG
authors.lastname: Vannucchi
authors.forename: Claudio
authors.initials: C
authors.lastname: Zardo
authors.forename: Letizia
authors.initials: L
authors.lastname: Corsani
authors.forename: Maria-Simonetta
authors.initials: MS
authors.lastname: Faussone-Pellegrini
mesh_terms: D000818:Animals; D015153:Blotting, Western; D003106:Colon / Q000737:chemistry / Q000166:cytology; D004064:Digestive System / Q000737:chemistry* / Q000166:cytology; D016189:Dystrophin / Q000378:metabolism*; D007082:Ileum / Q000737:chemistry / Q000166:cytology; D007150:Immunohistochemistry; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D009130:Muscle, Smooth / Q000737:chemistry* / Q000166:cytology; D009474:Neurons / Q000737:chemistry* / Q000166:cytology; D013270:Stomach / Q000737:chemistry / Q000166:cytology
chemical_list: D016189:Dystrophin