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As a master component of endosomal sorting complex required for transport proteins, hepato-cyte growth factor-regulated tyrosine kinase substrate (Hgs) participates multiple cellular be-haviors.However, the physiological role of Hgs in smooth muscle cells (SMCs) is by far unknown.Here we explored the in vivo function of Hgs in SMCs by using a conditional gene knockout strategy.Hgs deficiency in SMCs uniquely led to a progressive dilatation of esophagus with a remarkable thinning muscle layer.