TY - JOUR
T1 - Genetics of enteric neuropathies
AU - Brosens, Erwin
AU - Burns, Alan J.
AU - Brooks, Alice S.
AU - Matera, Ivana
AU - Borrego, Salud
AU - Ceccherini, Isabella
AU - Tam, Paul K.
AU - García-Barceló, Maria-Mercè
AU - Thapar, Nikhil
AU - Benninga, Marc A.
AU - Hofstra, Robert M. W.
AU - Alves, Maria M.
PY - 2016
Y1 - 2016
N2 - Abnormal development or disturbed functioning of the enteric nervous system (ENS), the intrinsic innervation of the gastrointestinal tract, is associated with the development of neuropathic gastrointestinal motility disorders. Here, we review the underlying molecular basis of these disorders and hypothesize that many of them have a common defective biological mechanism. Genetic burden and environmental components affecting this common mechanism are ultimately responsible for disease severity and symptom heterogeneity. We believe that they act together as the fulcrum in a seesaw balanced with harmful and protective factors, and are responsible for a continuum of symptoms ranging from neuronal hyperplasia to absence of neurons
AB - Abnormal development or disturbed functioning of the enteric nervous system (ENS), the intrinsic innervation of the gastrointestinal tract, is associated with the development of neuropathic gastrointestinal motility disorders. Here, we review the underlying molecular basis of these disorders and hypothesize that many of them have a common defective biological mechanism. Genetic burden and environmental components affecting this common mechanism are ultimately responsible for disease severity and symptom heterogeneity. We believe that they act together as the fulcrum in a seesaw balanced with harmful and protective factors, and are responsible for a continuum of symptoms ranging from neuronal hyperplasia to absence of neurons
U2 - https://doi.org/10.1016/j.ydbio.2016.07.008
DO - https://doi.org/10.1016/j.ydbio.2016.07.008
M3 - Article
C2 - 27426273
SN - 0012-1606
VL - 417
SP - 198
EP - 208
JO - Developmental biology
JF - Developmental biology
IS - 2
ER -