Related Experiment Video

Updated: Jan 14, 2026

In Situ Detection of Ribonucleoprotein Complex Assembly in the C. elegans Germline using Proximity Ligation Assay
08:56

In Situ Detection of Ribonucleoprotein Complex Assembly in the C. elegans Germline using Proximity Ligation Assay

Published on: May 5, 2020

6.3K

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
PubMed

Insights

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.

Keywords:
ADPKDDNAJB11GPS cleavagePolycystin-1SDF2SDF2L1polycystic kidney disease

More Related Videos

Identification of Nucleolar Factors During HIV-1 Replication Through Rev Immunoprecipitation and Mass Spectrometry09:38

Identification of Nucleolar Factors During HIV-1 Replication Through Rev Immunoprecipitation and Mass Spectrometry

Published on: June 26, 2019

8.5K
In Situ Monitoring of Transiently Formed Molecular Chaperone Assemblies in Bacteria, Yeast, and Human Cells08:58

In Situ Monitoring of Transiently Formed Molecular Chaperone Assemblies in Bacteria, Yeast, and Human Cells

Published on: September 2, 2019

7.4K

Related Concept Videos

Protein Complexes with Interchangeable Parts01:57

Protein Complexes with Interchangeable Parts

Groups of proteins may form a complex where each protein in this complex has a different role in the overall execution of the complex’s function. Often some of the proteins in the complex can be replaced by a closely related variant to give a complex that contains many of the same components yet is functionally distinct.
The SCF ubiquitin ligase is a protein complex of five individual proteins. This complex attaches ubiquitin to other target proteins to mark them for degradation. In order...
2.9K
Regulation of Nuclear Protein Sorting01:45

Regulation of Nuclear Protein Sorting

Nuclear protein sorting regulates nucleus composition and gene expression, crucial for determining the fate of a eukaryotic cell. Hence, the entry and exit of molecules across the nuclear envelope is a tightly controlled process. Nuclear protein sorting can be inhibited by one of the following ways: 1) masking cargo signal sequences, 2) modifying the nuclear receptor's affinity for cargo, 3) controlling the nuclear pore size, 4) retaining the cargo during its transit to the cytosol or the...
3.2K
RNA Polymerase II Accessory Proteins02:36

RNA Polymerase II Accessory Proteins

3.8K
RNA Polymerase II Accessory Proteins02:36

RNA Polymerase II Accessory Proteins

Proteins that regulate transcription can do so either via direct contact with RNA Polymerase or through indirect interactions facilitated by adaptors, mediators, histone-modifying proteins, and nucleosome remodelers. Direct interactions to activate transcription is seen in bacteria as well as in some eukaryotic genes. In these cases, upstream activation sequences are adjacent to the promoters, and the activator proteins interact directly with the transcriptional machinery. For example, in...
10.8K
Cytoskeletal Accessory Proteins01:13

Cytoskeletal Accessory Proteins

The cytoskeleton is an essential cell component that plays several structural and functional roles. However, the filaments that make up the cytoskeleton cannot function independently and depend on the accessory or ancillary proteins to effectively carry out their function. Accessory proteins associate with cytoskeletal filaments and their monomers, aiding filament formation and function. They also help in the cross-communication among cytoskeletal filaments. Cytoskeletal accessory proteins are...
3.9K
Nuclear Protein Sorting01:34

Nuclear Protein Sorting

Nuclear protein sorting is the selective trafficking of histones, polymerases, gene regulatory proteins into the nucleus and exporting RNAs and ribosomes to the cytosol. It is a tightly controlled process that regulates gene expression within a cell.
Proteins targeted to the nucleus carry nuclear localization signals or NLS recognized by import receptors in the cytosol. Similarly, proteins with nuclear export signals are recognized by export receptors. Import and export receptors are...
6.2K