Altered globotriaosylceramide accumulation and mucosal neuronal fiber density in the colon of the Fabry disease mouse model.
Martina MasottiCecilia DelpreteGiovanni DothelVincenzo DonadioRoberto Rimondini GiorginiJuan Manuel PoliteiRocco LiguoriMarco CapriniPublished in: Neurogastroenterology and motility : the official journal of the European Gastrointestinal Motility Society (2019)
The observed alterations are likely to be a leading cause of gut motor dysfunctions experienced by FD patients and imply that the α-Gal A (-/0) male mouse represents a reliable model for translational studies on enteropathic pain and GI symptoms in FD.