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Study reveals how cells destroy faulty proteins in cystic fibrosis

ectors -- extends the life of faulty CFTR proteins and thereby enables some of it to assume a functional shape.

The trash collectors newly identified by the Cyr group are two different ubiquitin ligases, proteins that specifically recognize misshaped regions of CFTR and tag them with a degradation signal known as ubiquitin. The ubiquitin tag tells the cell to destroy the marked CFTR, a process overseen by a destroyer called the proteasome. This trash system is known as the ubiquitin proteasome pathway.

"We've identified the trash collectors that recognize misfolded proteins, decide if they might be toxic to the cell and mark them with ubiquitin. The ubiquitinated proteins, including faulty CFTR, are then degraded or shredded by the proteasome," Cyr said.

"Understanding this pathway gives us greater insight into what's wrong with the protein -- information that will help people design better therapeutics for the disease," Cyr said.


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Source:University of North Carolina School of Medicine


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