Podoplanin hetero-insufficiency mice with inflammation in the jejunum demonstrates a good animal model of congenital protein-losing enteropathy.

We have identified the Podoplanin expression in rat jejunal villi. We aimed to investigate the relationship between the Podoplanin expression in jejunal villi and the pathogenesis of congenital protein-losing enteropathy (PLE), using the Podoplanin heterozygous knock-out (Pdpn-het KO) mice and aspirin-induced inflammation of the jejunum. In the Pdpn-het KO mice the reticular and complexes distribution of Podoplanin was observed in the jejunal villi, resulting in be swollen of lamina propria. To confirm the abnormal distribution of small lymph vessels in the jejunal villi, the LYVE-1 immunohistochemical expression was investigated. The expression of Podoplanin and LYVE-1 in the jejunum of the Pdpn-het KO mice exhibited a distinct pattern. The intravenous administration of Evans blue dye appeared quickly into the mesenteric lymph vessels and lymph nodes in the wild-type but not observed in Pdpn-het KO mice. In the Pdpn-het KO mice, aspirin-induced jejunal inflammation produced a significant leakage of the intravenous administration of FITC-albumin into the jejunal lumen. The hypoalbuminemia in the blood and the marked distribution of FITC-albumin in the jejunal villi were also observed in the mice. In conclusion, we proposed that Pdpn-het KO mice with jejunal inflammation demonstrates a good animal model of the congenital PLE.