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Two specific sulfatide species are dysregulated during renal development in a mouse model of Alport Syndrome
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June 13 2019
Source: Lipids. 54(6-7):411-418 -
Alternative Title:Lipids
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Description:Alport syndrome is caused by mutations in collagen IV that alter the morphology of renal glomerular basement membrane. Mutations result in proteinuria, tubulointerstitial fibrosis, and renal failure but the pathogenic mechanisms are not fully understood. Using imaging mass spectrometry, we aimed to determine whether the spatial and/or temporal patterns of renal lipids are perturbed during the development of Alport syndrome in the mouse model. Our results show that most sulfatides are present at similar levels in both the wild-type (WT) and the Alport kidneys, with the exception of two specific sulfatide species, SulfoHex-Cer(d18:2/24:0) and SulfoHex-Cer(d18:2/16:0). In the Alport but not in WT kidneys, the levels of these species mirror the previously described abnormal laminin expression in Alport syndrome. The presence of these sulfatides in renal tubules but not in glomeruli suggests that this specific aberrant lipid pattern may be related to the development of tubulointerstitial fibrosis in Alport disease.
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Pubmed ID:31197846
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Pubmed Central ID:PMC6655412
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Funding:R01 GM058008/NIGMS NIH HHS/National Institute of General Medical Sciences/United States ; R24 DK103067/NIDDK NIH HHS/National Institute of Diabetes and Digestive and Kidney Diseases/United States ; P41 GM103391/NIGMS NIH HHS/National Institute of General Medical Sciences/United States ; R01 DK018381/NIDDK NIH HHS/National Institute of Diabetes and Digestive and Kidney Diseases/United States ; R01 DK065138/NIDDK NIH HHS/National Institute of Diabetes and Digestive and Kidney Diseases/United States ; ... More +
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