Open AccessPhenotypic switch of smooth muscle cells in paediatric chronic intestinal pseudo‐obstruction syndrome
Author(s) -
Martire Delphine,
Garnier Sarah,
Sagnol Sébastien,
Bourret Annick,
Marchal Stéphane,
Chauvet Norbert,
Guérin Amandine,
Forgues Dominique,
Berrebi Dominique,
Chardot Christophe,
Bellaiche Marc,
Rendu John,
Kalfa Nicolas,
Faure Sandrine,
de Santa Barbara Pascal
Publication year - 2021
Publication title -
journal of cellular and molecular medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.44
H-Index - 130
eISSN - 1582-4934
pISSN - 1582-1838
DOI - 10.1111/jcmm.16367
Subject(s) - phenotype , biology , pdgfra , phenotypic switching , microbiology and biotechnology , motility , pathology , immunology , cancer research , genetics , gene , medicine , gist , stromal cell
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.