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Kidney Week

Abstract: FR-PO547

Involvement of Glycolipids in Cyst Formation in Cystic Kidney Disease

Session Information

Category: Genetic Diseases of the Kidneys

  • 1201 Genetic Diseases of the Kidneys: Cystic

Authors

  • Noda, Yuhei, Nagoya Daigaku Daigakuin Igakukei Kenkyuka Igakubu, Nagoya, Aichi, Japan
  • Kato, Noritoshi, Nagoya Daigaku Daigakuin Igakukei Kenkyuka Igakubu, Nagoya, Aichi, Japan
  • Sato, Yuka, Nagoya Daigaku Daigakuin Igakukei Kenkyuka Igakubu, Nagoya, Aichi, Japan
  • Maeda, Kayaho, Nagoya Daigaku Daigakuin Igakukei Kenkyuka Igakubu, Nagoya, Aichi, Japan
  • Kosugi, Tomoki, Nagoya Daigaku Daigakuin Igakukei Kenkyuka Igakubu, Nagoya, Aichi, Japan
  • Maruyama, Shoichi, Nagoya Daigaku Daigakuin Igakukei Kenkyuka Igakubu, Nagoya, Aichi, Japan
Background

ADPKD is an important disease that can lead to end-stage renal failure, yet many aspects of the mechanism of cyst enlargement remain unclear. In this study focusing on cystic kidney diseases, we examined the differences in glycolipids, a post-translational modification of proteins. Previous studies have demonstrated the accumulation of sphingolipids, such as GlcCer, LacCer, and GM3, in cystic kidney diseases. However, their direct involvement in cyst formation has not been established.

Methods

Glycolipids were extracted from the kidneys of PCK rats, a model of ARPKD, and developed by thin layer chromatography. Total RNA was extracted from the kidney tissue of the model animals and expression of glycosyltransferase was confirmed by qPCR. The same was also confirmed by WB.

Results

Thin layer chromatography of glycolipids extracted from renal tissue revealed changes in gangliosides including GM3, an acidic glycolipid. In cystic kidney tissue, GD3 with malignant phenotype was detected. qPCR and WB showed decreased expression of B4galnt1, a glycosyltransferase, in the medullary tubules of cystic kidney tissue. Primary culture of kidney cells from ADPKD patients also showed decreased expression of B4GALNAT1.

Conclusion

In the kidneys of mouse models of cystic kidney disease, disturbance in the composition of glycolipids was observed. Accumulation of GD3 is said to be involved in signaling of cell proliferation and may contribute to cyst enlargement. Decreased expression of B4galnt1 could explain these disturbed glycolipids change.