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Abstract: TH-PO735

Lower NBL1 Increases Kidney Function in a Glomerular Damage Model

Session Information

Category: Glomerular Diseases

  • 1403 Podocyte Biology

Authors

  • Bufi, Rei, The Jackson Laboratory, Bar Harbor, Maine, United States
  • Sheehan, Susan Marie, The Jackson Laboratory, Bar Harbor, Maine, United States
  • Korstanje, Ron, The Jackson Laboratory, Bar Harbor, Maine, United States
Background

Increased serum levels of neuroblastoma suppressor of tumorigenicity 1 (NBL1) have been associated with a faster decline in kidney function in patients with diabetic nephropathy (DN), and in vitro experiments suggest higher NBL1 levels lead to podocyte apoptosis. We aim to test whether there is a causal relationship between NBL1 and CKD by manipulating Nbl1 expression.

Methods

We generated an Nbl1 heterozygous knockout (HET) model in the C57BL/6J background (homozygous knockout is not viable) and confirmed lower NBL1 levels in these mice. We tested two kidney damage models. In the first model, we induced tubular damage using a low-dose cisplatin protocol. In the second model, we induced glomerular damage by introducing a Col4a5 mutation through breeding.

Results

In our tubular damage model, we did not find differences in renal phenotypes between the HET mice and wildtype (WT) littermates. Bulk RNA-seq also showed no differences between the two groups.
However, in our glomerular damage model, HET mice have a higher glomerular filtration rate than their WT littermates. The kidneys show no difference in glomerular damage at the gross histological level, but a more detailed study of the podocytes is ongoing. In addition, bulk RNA-seq showed approximately 130 differentially expressed genes between the two groups, including Jun, Junb, Fos, Fosb, Egr1, Egr2, and Egr3, which encode transcription factors implicated in the pathogenesis of DN.

Conclusion

Our preliminary results suggest that NBL1 is causal for decreased kidney function only in the model defined by glomerular pathology. Our next steps aim to determine what drives increased NBL1 expression, and the source of serum NBL1.