Game changer for cystic fibrosis? Discovery of new cell could alter quest for treatment

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Juliana Eberschlag, a cystic fibrosis patient. Image credit: Andrew Francis Wallace

The discovery of a new type of cell could fundamentally alter how cystic fibrosis researchers seek a cure for the often-fatal hereditary disease. A paper published [August 1] in Nature describes how an ongoing cellular mapping project helped scientists identify this rare cell type, which appears to play a key role in managing rehydration and pH balance—huge issues for cystic fibrosis patients.

Scientists have long known mucus proteins do not form properly in cystic fibrosis patients, and that these structural irregularities cause salt–water imbalances and dehydration. This leads to a buildup in the lungs of thick, sticky mucus that can trigger breathing problems and potentially life-threatening infections.

The newly identified cells have been dubbed “pulmonary ionocytes” because they seem to be involved in transporting ions—electrically charged particles like those in table salt—across lung tissue. In cystic fibrosis patients these cells also appear to produce vast quantities of mutated mucus proteins, which are called cystic fibrosis transmembrane conductance regulators (CFTR).

Related article:  'Short sleepers': People with this genetic mutation need less sleep than the rest of us

How could such a fundamental thing have been missed for so long? “I think it just speaks to the complexity of the disease and the fact that we really just didn’t understand the cellular basis of the lung,” says [pumonologist] Jayaraj Rajagopal.

Rajagopal thinks this work will lead to many more surprises, and to a far deeper understanding of both healthy and diseased cells.

Read full, original post: Newly Discovered Cell Type May Fuel Cystic Fibrosis

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