How polycystin proteins in kidney cell cilia control electrical signals
Deciphering polycystin-dependent electric signaling of primary cilia in the renal system
This work looks at how mutations in polycystin proteins change electrical signals in tiny antenna-like parts of kidney cells, which matters for people with ADPKD.
Quick facts
| Grant type | NIH-funded research |
|---|---|
| Study type | NIH-funded research |
| Funding institution | University of Pittsburgh at Pittsburgh NIH-funded |
| Lab location | 1 site (Pittsburgh, United States) |
| Project ID | NIH-11496834 on NIH RePORTER |
What this research studies
Researchers are studying the tiny antenna-like structures (primary cilia) on kidney cells to see how polycystin proteins control ion flow and electrical signaling. They will use a specialized lab technique called ciliary patch-clamp to record channel activity directly from the ciliary membrane. The project focuses on ADPKD-causing changes in the PC-1 protein, especially alterations in its N-terminal C-type lectin-like domain, and may use gene-editing and cell models to reproduce those mutations. The goal is to clarify the basic electrical defect that contributes to cyst formation in ADPKD and point toward targets for future therapies.
Who could benefit from this research
Good fit: People with autosomal dominant polycystic kidney disease, especially those with known PKD1 or PKD2 mutations, are the most relevant group for these findings.
Not a fit: Patients without ADPKD or whose kidney disease has a different cause are unlikely to see direct benefit from this basic laboratory work.
Why it matters
Potential benefit: If successful, this work could reveal why specific genetic mutations cause ADPKD and help guide development of targeted treatments to protect kidney function.
How similar studies have performed: Prior laboratory work has linked the PC-1 N-terminal domain to channel activation, but directly recording ciliary channel changes caused by ADPKD mutations is relatively novel.
Where this research is happening
Pittsburgh, United States
- University of Pittsburgh at Pittsburgh — Pittsburgh, United States (Active)
Researchers
- Principal investigator: Ha, Kotdaji — University of Pittsburgh at Pittsburgh
- Study coordinator: Ha, Kotdaji
About this research
- This is an active NIH-funded research project — typically early-stage science, not a clinical trial accepting patient enrollment.
- Some NIH-funded labs run parallel clinical studies or seek volunteers for related work. To check, contact the principal investigator or institution listed above.
- For full project details, budget, and progress reports, visit the official NIH RePORTER page below.