The Scotsman

Immune cells find raises hope for cystic fibrosis treatments

- By HILARY DUNCANSON

Fresh insights into how cystic fibrosis affects immune cells could pave the way for new treatments for the condition, Scots researcher­s believe.

Scientists have discovered that cells in patients with cystic fibrosis that normally defend against infection can also perpetuate damage to the lungs.

Drugs that target these cells could help to stem progressio­n of the disease, the scientists claimed.

A team at the University of Edinburgh, working with researcher­s in the US and Ireland, focused on immune cells known as neutrophil­s –part of the body’s first line of defence against infections.

Once an infection has been cleared, neutrophil­s are usually programmed to die off quietly, so that they do not mistakenly cause damage to healthy tissues.

However, in patients with cystic fibrosis, neutrophil­s survive longer than they are supposed to and are a key contributo­r to the lung damage associated with the condition, the experts said.

The team discovered that neutrophil­s from cystic fibrosis patients are more resistant to the usual mechanism of cell death – a process called apoptosis – with their ability to survive longer directly related to the underlying genetic mutation that causes cystic fibrosis.

Instead, the cells die by a different process, which causes them to disintegra­te and expel their damaging contents into the surroundin­g area of the lung, the team said.

This process promotes inflammati­on and may therefore promote damage to healthy tissues in the lung.

The researcher­s said they were able to block this process by treatment with a drug that encourages neutrophil­s to die by apoptosis, paving the way for new therapies for the disease.

Dr Robert Gray, of the Medical Research Council Centre for Inflammati­on Research at Edinburgh University, said: “Therapies targeting inflammati­on are not readily available but are needed for the treatment of cystic fibrosis.

“This work, although at an early stage, will help in the developmen­t of new antiinflam­matory treatments for this debilitati­ng condition.”

Cystic fibrosis causes thick mucus to build up in patient’s lungs and digestive tracts. It causes persistent coughs and breathing difficulti­es, as well as leaving patients vulnerable to recurrent infections.

Around 11,000 people in the UK are currently living with cystic fibrosis.

The research, published in the journal Thorax, was funded by the Medical Research Council and Wellcome.

 ??  ?? 0 The team said the discovery could lead to better treatments
0 The team said the discovery could lead to better treatments

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