Search

In this study, we examined the role of autophagy in the initiation of lipid increases in renal epithelial HK2 cells. We found that trivalent chromium [Cr(III)] induced autophagy by activating sphingomyelin phosphodiesterase 2 (SMPD2). SMPD2 increases levels of ceramide and other lipids. Confocal immunofluorescence microscopy showed that signals of ceramide overlapped with LC3, suggesting that ceramide might play an important role in the formation of autophagosome. In conclusion, our data indicate that Cr(III) induces autophagy via structural aberration of organelle membrane, in particular by the increase of lipid compositions in addition to autophagy-associated proteins. This article is protected by copyright. All rights reserved

Congenital erythropoietic porphyria (CEP), or “Gunther disease”, is a rare variant of porphyria. It is an autosomal recessive disease caused by deficient uroporphyrinogen III synthase (URO-III-synthase), the fourth enzyme in the heme biosynthetic pathway. We herein report a case of a man with the typical clinical presentations of hyper- and hypo-pigmentation and blister formation over sun-exposed areas, mutilation of the fingers, dark-purple urine, and erythrodontia with pinkish fluorescence under a Wood’s lamp. The diagnosis was confirmed by decreased activity of URO-III-synthase in red blood cells (RBC) and a porphyrin profile compatible with CEP.

Background: Neurofibroma consists of abundant extracellular matrix and many types of cells, including Schwann cells (SCs), mast cells (MCs), fibroblasts and endothelial cells. As SCs have been found to be the cell of origin for neurofibroma, how MCs may migrate into the tumor has not been fully clarified. Given that chemokine receptor CCR3 is found predominantly expressed by differentiated MCs, we postulated that CCR3 may play a role in the homing of MCs to neurofibroma. The goal of this study is to investigate the possible involvement of chemokine receptor CCR3 in the migration of MCs to the neurofibroma. Methods: Expressional and functional assays for CCR3 and its ligands were performed on MCs and SCs. Results: By real-time quantitative polymerase chain reaction and enzyme-linked immunosorbent assay, we found one of the CCR3 ligand, CCL7 was highly expressed by murine SC cell line SW10, and also moderately expressed by MCs. In serial chemotaxis assays, MCs were found specifically responsive to CCL7 and also condition medium from SW10 cells, indicating SCs may attract MCs by CCR3-mediated cell migration. Conclusion: The interaction of CCR3 and CCL7 may play important roles for MC migration toward SC in the neurofibroma.