SHROOM family members play diverse roles in the development of the nervous system and other tissues. SHROOM1 is a PDZ domain-containing actin-binding protein that is required for neural tube morphogenesis. It facilitates neurulation by regulating the morphology of neurepithelial cells via the apical positioning of an actomyosin network in the neurepithelium. SHROOM1 is also expressed in pulmonary arterial smooth muscle cells and its expression is significantly decreased in mouse and human models of pulmonary arterial hypertension, suggesting that there may be a link between SHROOM1 expression and pulmonary arterial hypertension.