Abnormal distribution of sarcoglycan subcomplex in colonic smooth muscle cells of aganglionic bowel

  • Authors:
    • Salvatore Arena
    • Giuseppina Cutroneo
    • Angelo Favaloro
    • Maria Teresa Sinatra
    • Fabio Trimarchi
    • Silvia Scarvaglieri
    • Agostino Mallamace
    • Francesco Arena
    • Giuseppe Anastasi
    • Vincenzo Di Benedetto
  • View Affiliations

  • Published online on: March 1, 2010     https://doi.org/10.3892/ijmm_00000352
  • Pages: 353-359
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Abstract

Hirschsprung's disease (HD) is a development disorder of the enteric nervous system in which the altered innervation explains the inability of the aganglionic segment to relax. Impairment of cytoskeleton in SMC of aganglionic bowel has been shown. Sarcoglycan subcomplex (SG) may support the development and maintenance of muscle cells. We examined the SG subunit expression in colonic aganglionic and ganglionic specimens obtained from patients with HD. Full-thickness bowel specimens were obtained from six patients with HD. Six normal colon specimens were used as controls. Immunofluorescent analysis and reverse transcriptase polymerase chain reaction evaluation were performed for α-, β-, γ-, δ- and ε-SG. In control colon, the indirect immunofluorescence showed a strong staining pattern of β- γ- δ- and ε-SG while a weak positivity of α-SG was recorded. In aganglionic bowel, immunofluorescence intensity values documented a significant lack of ε-SG while an enhanced α-SG, coupled to a loss of ε-SG, was recorded in ganglionic bowel in HD-affected patients. Our observations underscore the assumption that non-neuronal elements of the colon might play a key role in the pathogenesis of HD and loss of ε-SG might critically alter the cytoskeleton in the aganglionic bowel segment. Up-regulation of α-SG is probably an acquired phenomenon to reinforce the sarcolemma and to perform a forceful contraction in dilated ganglionic HD-affected colon, related to chronic pseudo-obstruction, contributing to the intestinal dysmotility that persists in 20% of patients after resection of the aganglionic bowel.

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March 2010
Volume 25 Issue 3

Print ISSN: 1107-3756
Online ISSN:1791-244X

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Spandidos Publications style
Arena S, Cutroneo G, Favaloro A, Sinatra MT, Trimarchi F, Scarvaglieri S, Mallamace A, Arena F, Anastasi G, Di Benedetto V, Di Benedetto V, et al: Abnormal distribution of sarcoglycan subcomplex in colonic smooth muscle cells of aganglionic bowel. Int J Mol Med 25: 353-359, 2010
APA
Arena, S., Cutroneo, G., Favaloro, A., Sinatra, M.T., Trimarchi, F., Scarvaglieri, S. ... Di Benedetto, V. (2010). Abnormal distribution of sarcoglycan subcomplex in colonic smooth muscle cells of aganglionic bowel. International Journal of Molecular Medicine, 25, 353-359. https://doi.org/10.3892/ijmm_00000352
MLA
Arena, S., Cutroneo, G., Favaloro, A., Sinatra, M. T., Trimarchi, F., Scarvaglieri, S., Mallamace, A., Arena, F., Anastasi, G., Di Benedetto, V."Abnormal distribution of sarcoglycan subcomplex in colonic smooth muscle cells of aganglionic bowel". International Journal of Molecular Medicine 25.3 (2010): 353-359.
Chicago
Arena, S., Cutroneo, G., Favaloro, A., Sinatra, M. T., Trimarchi, F., Scarvaglieri, S., Mallamace, A., Arena, F., Anastasi, G., Di Benedetto, V."Abnormal distribution of sarcoglycan subcomplex in colonic smooth muscle cells of aganglionic bowel". International Journal of Molecular Medicine 25, no. 3 (2010): 353-359. https://doi.org/10.3892/ijmm_00000352