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SDF2 and SDF2L1 are essential co-factors of DNAJB11 for Polycystin-1 processing
Tilman Busch1, Björn Neubauer1, Sophia Sediq1
1Department of Medicine IV - Nephrology and Primary Care, Faculty of Medicine and Medical Center, University of Freiburg, Freiburg, Germany.
The Journal of Biological Chemistry
|October 18, 2025
Summary
DNAJB11 mutations cause kidney disease by impairing Polycystin-1 (PC1) processing. This study identifies SDF2 and SDF2L1 as key DNAJB11 complex members essential for normal PC1 processing and kidney function.
Area of Science:
- Molecular Biology
- Cell Biology
- Genetics
Background:
- Mutations in DNAJB11, a co-chaperone, are linked to polycystic kidney disease.
- The disease mechanism involves impaired processing of Polycystin-1 (PC1), a protein central to autosomal dominant polycystic kidney disease (ADPKD).
- Chaperones typically function in multiprotein complexes to aid protein processing, but DNAJB11's complex partners for PC1 processing were unknown.
Purpose of the Study:
- To identify DNAJB11-interacting proteins involved in PC1 processing.
- To elucidate the role of these interacting proteins in the molecular mechanism of DNAJB11-related kidney disease.
Main Methods:
- Unbiased interaction proteomics screen to identify DNAJB11-binding proteins.
- Generation and utilization of knockout cell lines for DNAJB11, SDF2, and SDF2L1.
- Analysis of protein abundance and Polycystin-1 (PC1) processing in wild-type and knockout cells.
Main Results:
- SDF2 and SDF2L1 were identified as strong interaction partners of DNAJB11.
- Loss of SDF2 and SDF2L1 phenocopied the PC1 processing defect observed in DNAJB11-deficient cells.
- Reciprocal interdependence of DNAJB11, SDF2, and SDF2L1 protein levels was demonstrated.
Conclusions:
- SDF2 and SDF2L1 are essential subunits of the DNAJB11 complex.
- This complex is crucial for the proper processing of Polycystin-1 (PC1).
- The findings provide critical insights into the molecular basis of DNAJB11-related polycystic kidney disease.
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