Phenotypic switch of smooth muscle cells in paediatric chronic intestinal pseudo-obstruction syndrome.
PDGFR pathway
chronic intestinal pseudo-obstruction Disease
intestinal motility disorders
plasticity
smooth muscle cells
Journal
Journal of cellular and molecular medicine
ISSN: 1582-4934
Titre abrégé: J Cell Mol Med
Pays: England
ID NLM: 101083777
Informations de publication
Date de publication:
04 2021
04 2021
Historique:
revised:
31
12
2020
received:
18
03
2020
accepted:
12
01
2021
pubmed:
4
3
2021
medline:
23
9
2021
entrez:
3
3
2021
Statut:
ppublish
Résumé
Smooth Muscle Cells (SMC) are unique amongst all muscle cells in their capacity to modulate their phenotype. Indeed, SMCs do not terminally differentiate but instead harbour a remarkable capacity to dedifferentiate, switching between a quiescent contractile state and a highly proliferative and migratory phenotype, a quality often associated to SMC dysfunction. However, phenotypic plasticity remains poorly examined in the field of gastroenterology in particular in pathologies in which gut motor activity is impaired. Here, we assessed SMC status in biopsies of infants with chronic intestinal pseudo-obstruction (CIPO) syndrome, a life-threatening intestinal motility disorder. We showed that CIPO-SMCs harbour a decreased level of contractile markers. This phenotype is accompanied by an increase in Platelet-Derived Growth Factor Receptor-alpha (PDGFRA) expression. We showed that this modulation occurs without origin-related differences in CIPO circular and longitudinal-derived SMCs. As we characterized PDGFRA as a marker of digestive mesenchymal progenitors during embryogenesis, our results suggest a phenotypic switch of the CIPO-SMC towards an undifferentiated stage. The development of CIPO-SMC culture and the characterization of SMC phenotypic switch should enable us to design therapeutic approaches to promote SMC differentiation in CIPO.
Identifiants
pubmed: 33656779
doi: 10.1111/jcmm.16367
pmc: PMC8051695
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
4028-4039Subventions
Organisme : Agence Nationale de la Recherche
ID : ANR-17-CE14-0043-01
Organisme : Association patient POIC
Organisme : Association Française contre les Myopathies
ID : 20467
Organisme : Association Française contre les Myopathies
ID : 20749
Organisme : FIMATHO
Informations de copyright
© 2021 The Authors. Journal of Cellular and Molecular Medicine published by Foundation for Cellular and Molecular Medicine and John Wiley & Sons Ltd.
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