Understanding how cells manage proteins to prevent disease
Regulation and Mechanisms of ER Proteostasis
This research explores how our cells clean up faulty proteins, which is important for conditions like Cystic Fibrosis and AAT deficiency.
Quick facts
| Grant type | NIH-funded research |
|---|---|
| Study type | NIH-funded research |
| Funding institution | University of Iowa NIH-funded |
| Lab location | 1 site (Iowa City, United States) |
| Project ID | NIH-11131174 on NIH RePORTER |
What this research studies
Our cells have a special compartment called the endoplasmic reticulum (ER) that makes sure proteins fold correctly. When proteins don't fold right, the ER has two main ways to get rid of them: ER-associated degradation (ERAD) and ER-autophagy (ER-phagy). This project aims to understand how cells decide which cleanup method to use and how these processes differ in various cell types. By studying these mechanisms, we hope to learn more about why faulty proteins build up in certain diseases.
Who could benefit from this research
Good fit: This foundational research is most relevant to patients with conditions like Cystic Fibrosis, Alpha-1 Antitrypsin (AAT) deficiency, or Niemann-Pick disease type C1, where protein misfolding is a key problem.
Not a fit: Patients whose conditions are not related to protein misfolding or ER quality control are unlikely to see direct benefits from this specific research.
Why it matters
Potential benefit: If successful, this work could lead to new ways to treat diseases caused by misfolded proteins by improving how cells clear them out.
How similar studies have performed: While the specific details of how cells choose between ERAD and ER-phagy are still being uncovered, the general concepts of protein quality control and degradation pathways are well-established in scientific research.
Where this research is happening
Iowa City, United States
- University of Iowa — Iowa City, United States (Active)
Researchers
- Principal investigator: Schultz, Mark Louis — University of Iowa
- Study coordinator: Schultz, Mark Louis
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.