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Post-translational modifications of the polycystin proteins
1Kidney Genetics Group, Academic Nephrology Unit, University of Sheffield Medical School, Sheffield, UK.
Cellular Signalling
|April 23, 2020
Summary
Autosomal dominant polycystic kidney disease (ADPKD), a common inherited kidney failure cause, involves PKD1/PKD2 gene mutations. Understanding polycystin protein post-translational modifications (PTMs) is crucial for future ADPKD research and potential treatments.
Area of Science:
- Nephrology
- Genetics
- Molecular Biology
Background:
- Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent inherited kidney disorder, affecting millions globally.
- It is primarily caused by germline mutations in the PKD1 or PKD2 genes, encoding polycystin-1 (PC1) and polycystin-2 (PC2) proteins.
Purpose of the Study:
- To review the role of post-translational modifications (PTMs) in regulating polycystin function in ADPKD.
- To highlight the significance of PTMs in polycystin trafficking, signaling, localization, and stability.
Main Methods:
- Literature review of studies investigating PTMs of PC1 and PC2.
- Analysis of reported PTMs including phosphorylation, glycosylation, and proteolytic cleavage.
Main Results:
- Several PTMs of PC1 and PC2 have been identified and characterized.
- Specific PTMs are known to influence polycystin function and cellular localization.
- The precise functional impact of all identified PTMs requires further elucidation.
Conclusions:
- Post-translational modifications play a critical role in the physiological function of polycystins.
- Further research is needed to fully understand the functional significance of individual PTMs in ADPKD.
- Mutations disrupting PTMs may contribute to cystogenesis, though more evidence is required.
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