Phenotypic switch of smooth muscle cells in paediatric chronic intestinal pseudo‐obstruction syndrome

Smooth Muscle Cells (SMC) are unique amongst all muscle cells in their capacity to modulate phenotype. Indeed, SMCs do not terminally differentiate but instead harbour a remarkable dedifferentiate, switching between quiescent contractile state and highly proliferative migratory phenotype, quality often associated SMC dysfunction. However, phenotypic plasticity remains poorly examined the field of gastroenterology particular pathologies which gut motor activity is impaired. Here, we assessed status biopsies infants with chronic intestinal pseudo-obstruction (CIPO) syndrome, life-threatening motility disorder. We showed that CIPO-SMCs decreased level markers. This phenotype accompanied by an increase Platelet-Derived Growth Factor Receptor-alpha (PDGFRA) expression. this modulation occurs without origin-related differences CIPO circular longitudinal-derived SMCs. As characterized PDGFRA as marker digestive mesenchymal progenitors during embryogenesis, our results suggest switch CIPO-SMC towards undifferentiated stage. The development culture characterization should enable us design therapeutic approaches promote differentiation CIPO.