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- W1570173586 abstract "Fabry disease is a lysosomal storage disorder caused by deficiency of α-galactosidase A (α-gal A), resulting in deposition of globotriaosylceramide (Gb3) in the vascular endothelium. Deacylated Gb3, globotriaosylsphingosine (lyso-Gb3), also accumulated in the tissues of Fabry disease patients. Lyso-Gb3 has been suggested as a candidate biomarker. According to renal and cardiac pathological findings, epithelial-to-mesenchymal transition (EMT) could be a possible pathogenic mechanism in Fabry disease. However, the association of EMT with Fabry disease has not been elucidated yet. Expression level of EMT markers such as E-cadherin, N-cadherin, β-catenin and α-SMA in mouse renal glomerular mesangial cell (SV40MES13) significantly increased when treated with Gb3. Whereas, in human proximal renal tubular epithelial cells (HK-2), expression of EMT markers were significantly altered when treated with lyso-Gb3 rather than treated with Gb3. Additionally, we observed that the activation of TGF-β, phospho-AKT, and PI3K were highly elevated in these cells. When we treated recombinant α-gal A with Gb3 or lyso-Gb3, TGF-β expression was reversed in HK2 cells. Our study demonstrates that Gb3 and lyso-Gb3 can potentiate EMT-like processes through AKT/PI3K signaling pathway in HK2 and SV40MES13 cells. Moreover, these findings suggest that lyso-Gb3, rather than Gb3 may be a crucial role nephropathy of Fabry disease by inducing EMT and may contribute to a better understanding of the renal fibrosis." @default.
- W1570173586 created "2016-06-24" @default.
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- W1570173586 date "2014-04-01" @default.
- W1570173586 modified "2023-09-25" @default.
- W1570173586 title "Globotriaosylceramide and globotriaosylsphingosine mediated epithelial‐to‐mesenchymal transition in kidney cells: implication for Fabry disease nephropathy (600.1)" @default.
- W1570173586 doi "https://doi.org/10.1096/fasebj.28.1_supplement.600.1" @default.
- W1570173586 hasPublicationYear "2014" @default.
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