Jeffrey Spraggins
Last active: 3/18/2020

Two Specific Sulfatide Species Are Dysregulated during Renal Development in a Mouse Model of Alport Syndrome.

Gessel MM, Spraggins JM, Voziyan PA, Abrahamson DR, Caprioli RM, Hudson BG
Lipids. 2019 54 (6-7): 411-418

PMID: 31197846 · PMCID: PMC6655412 · DOI:10.1002/lipd.12171

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.

© 2019 AOCS.

MeSH Terms (7)

Animals Disease Models, Animal Kidney Tubules Lipid Metabolism Mice Nephritis, Hereditary Sulfoglycosphingolipids

Connections (2)

This publication is referenced by other Labnodes entities:

Links