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Updated: May 29, 2026
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Comparative Proteomic Analysis of Whole Kidney, Medulla, and Cortical Tubules in Diabetic Pathogenesis of Kidney Injury in Mice
Published on: May 2, 2025
Glycosphingolipids and kidney disease.
Andrew R Mather1, Leah J Siskind
1Department of Medicine, Division of General Internal Medicine/Geriatrics, Medical University of South Carolina, Charleston, South Carolina, USA.
Advances in Experimental Medicine and Biology
|September 13, 2011
Summary
Glycosphingolipids are crucial for kidney function and are implicated in various kidney diseases, including Fabry
Area of Science:
- Biochemistry
- Nephrology
- Lipid Metabolism
Background:
- Glycosphingolipids, formed by adding sugar moieties to ceramide, are abundant in the kidney.
- Defects in glycosphingolipid metabolism, like in Fabry disease due to alpha-galactosidase A deficiency, lead to lipid accumulation and kidney pathology.
- Emerging evidence links glycosphingolipids to diverse kidney conditions such as polycystic kidney disease, acute kidney injury, and diabetic nephropathy.
Purpose of the Study:
- To review the mechanistic role of glycosphingolipids in kidney disease.
- To discuss the involvement of glycosphingolipids in kidney disease associated with metabolic syndrome.
- To highlight the therapeutic potential of targeting glycosphingolipid metabolism.
Main Methods:
- Literature review of studies on glycosphingolipids and kidney disease.
- Analysis of data from inherited lipid storage diseases (e.g., Fabry disease).
- Examination of evidence linking glycosphingolipids to metabolic syndrome-related kidney conditions.
Main Results:
- Inactivation of alpha-galactosidase A causes glycosphingolipid accumulation, leading to end-stage kidney disease.
- Glycosphingolipids play a role in polycystic kidney disease, acute kidney injury, glomerulonephritis, diabetic nephropathy, and kidney cancer.
- Inhibitors of glycosphingolipid synthesis show good tolerability in preclinical and clinical studies.
Conclusions:
- Altered renal glycosphingolipid metabolism is mechanistically linked to kidney disease.
- Targeting glycosphingolipid synthesis presents a promising therapeutic strategy for various kidney diseases.
- Further understanding of these lipid pathways can unlock new treatments for renal pathologies.
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