According to News Medical, scientific experiments examining what happens to the faulty channel protein that causes cystic fibrosis (CF) during inflammation have yielded unexpected results. In the article “An unexpected effect of TNF-a on F508del-CFTR maturation and function,” the authors of the study report that a 10-minute exposure of cells expressing the faulty CFTR channel to TNF-a, an inflammatory cytokine produced by white blood cells, can temporarily fix these cells.
The researchers found a transient form of this effect in HeLa cells, which the scientists found surprising. The researchers then repeated the experiments in human bronchial epithelial cells, which have more clinical relevance to the condition, and found an even better effect with the cells working as they should for at least 24 hours, according to News Medical. Additional experiments explained that TNF-a achieves this by preventing the faulty CFTR protein from being degraded before it is successfully transported to the cell membrane.
Aleksander Edelman, principal investigator of the study, says, “We hope to investigate whether TNF-? is linked to the severity of cystic fibrosis; if so then TNF-alpha levels might be one of a panel of molecules that could be used as a prognostic marker in the disease.”
The study has just passed peer review on F1000Reserach, an open science-publishing platform.
“These findings are an exciting development in the bid to find a cure for cystic fibrosis – a devastating disease that affects many thousands of people worldwide,” explains Rebecca Lawrence, managing director of F1000Research. “It is really important that results of this nature are published in the public domain as quickly as possible for peer review and we are pleased to be able to facilitate this through our open science publishing platform.”
Source: News Medical