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Kidney Week

Abstract: FR-PO0677

Spatial Transcriptomics Reveals New ADPKD-Specific Genes

Session Information

Category: Genetic Diseases of the Kidneys

  • 1201 Genetic Diseases of the Kidneys: Monogenic Kidney Diseases

Authors

  • Hidaka, Sumi, Shonan Kamakura General Hospital, Kidney Disease and Transplant Center, Kamakura, Japan
  • Salybekov, Amankeldi, Shonan Kamakura General Hospital, Kidney Disease and Transplant Center, Kamakura, Japan
  • Mochida, Yasuhiro, Shonan Kamakura General Hospital, Kidney Disease and Transplant Center, Kamakura, Japan
  • Ohtake, Takayasu, Shonan Kamakura General Hospital, Kidney Disease and Transplant Center, Kamakura, Japan
  • Kobayashi, Shuzo, Shonan Kamakura General Hospital, Kidney Disease and Transplant Center, Kamakura, Japan
Background

Autosomal dominant polycystic kidney disease (ADPKD) represents the most prevalent genetic cause of end-stage renal disease, characterized by the progressive enlargement of renal cysts.

Methods

To elucidate the cellular composition and underlying pathomechanisms driving ADPKD progression, we conducted spatial transcriptomic analyses on human ADPKD kidney samples.

Results

Our findings revealed significant infiltration of circulating immune B cell subsets into regions surrounding both small and large cysts, accompanied by inflammatory monocytes and macrophages, particularly M1 macrophages. Cell-to-cell interaction analysis using CellChat demonstrated robust interactions between endothelial cells, fibroblasts, and leukocytes, including B cells and macrophages. Further cell annotation identified five previously uncharacterized cell clusters located at the borders of small and large cysts. Among these, the largest unidentified cell cluster exhibited high expression of 10 specific unique genes, including KRT17, SFN, KRT7, SLPI, TACSTD2, ITGB6, MMP7, COL1A1, KRT19, CLDN4, and NNMT. Notably, these genes were predominantly expressed in cells forming trabeculae or septa within cysts. Gene ontology (GO) biological process analysis revealed enrichment in processes such as regulation of cell migration (GO:0030334, P < 2.124 × 10-14) within these clusters. Pathway analysis further highlighted the involvement of TNFα signaling via NFκB, epithelial-mesenchymal transition, and apoptosis as the primary drivers of disease progression. Interestingly, psudotime and entropy analysis demonstrated that cyst cell-specific genes more robust in distal convoluted tubule. The SLPI gene, which is not typically expressed in kidney tissue under normal conditions, was markedly upregulated in cystic cells, potentially contributing to increased fluid secretion.

Conclusion

In summary, our research identifies novel cyst-specific genes that regulate cyst cell growth and their transformation into fibrotic tissue through inflammatory and migration pathways, providing new insights into ADPKD pathogenesis.

Digital Object Identifier (DOI)