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The ablation of Cavin1 in rodents also causes a significant deviation from Mendelian ratio at weaning in a background\u2010dependent manner, suggesting the presence of undiscovered functions of Cavin1. In the current study, the results show that Cavin1 deficiency causes neonatal death in C57BL\/6J mice by dampening the storage and mobilization of glycogen in the liver, which leads to lethal neonatal hypoglycemia. Further investigation by electron microscopy reveals that Cavin1 deficiency impairs the fenestration in liver sinusoidal endothelial cells (LSECs) and impacts the permeability of endothelial barrier in the liver. Mechanistically, Cavin1 deficiency inhibits the RhoA\u2010Rho\u2010associated protein kinase 2\u2010LIM domain kinase\u2010Cofilin signaling pathway and suppresses the dynamics of the cytoskeleton, and eventually causes the reduction of fenestrae in LSECs. In addition, the defect of fenestration in LSECs caused by Cavin1 deficiency can be rescued by treatment with the F\u2010actin depolymerization reagent latrunculin A. In summary, the current study reveals a novel function of Cavin1 on fenestrae formation in LSECs and liver glycogen metabolism, which provide an explanation for the neonatal death of Cavin1 null mice and a potential mechanism for metabolic disorders in patients with Cavin1 mutation.<\/jats:p>","DOI":"10.1002\/advs.202000963","type":"journal-article","created":{"date-parts":[[2020,8,21]],"date-time":"2020-08-21T10:00:15Z","timestamp":1598004015000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Cavin1 Deficiency Causes Disorder of Hepatic Glycogen Metabolism and Neonatal Death by Impacting Fenestrations in Liver Sinusoidal Endothelial Cells"],"prefix":"10.1002","volume":"7","author":[{"given":"Zhuang","family":"Wei","sequence":"first","affiliation":[{"name":"State Key Laboratory of Genetic Engineering and School of Life Sciences Human Phenome Institute Fudan University  Shanghai 200433 China"},{"name":"Key Laboratory of Systems Biology Innovation Center for Cell Signaling Network CAS Center for Excellence in Molecular Cell Science Institute of Biochemistry and Cell Biology Shanghai Institutes for Biological Sciences CAS  320 Yueyang Road Shanghai 200031 China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jigang","family":"Lei","sequence":"additional","affiliation":[{"name":"Key Laboratory of Systems Biology Innovation Center for Cell Signaling Network CAS Center for Excellence in Molecular Cell Science Institute of Biochemistry and Cell Biology Shanghai Institutes for Biological Sciences CAS  320 Yueyang Road Shanghai 200031 China"},{"name":"The Department of Biology Tongji University  Shanghai 200092 China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Feng","family":"Shen","sequence":"additional","affiliation":[{"name":"Department of Hepatobiliary Surgery Dongfeng Hospital Hubei University of Medicine  Shiyan Hubei 442001 China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuxiang","family":"Dai","sequence":"additional","affiliation":[{"name":"Department of Cardiology Zhongshan Hospital Fudan University Shanghai Institute of Cardiovascular Disease  Shanghai 200031 P. 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