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Cystic Fibrosis Trust

Making mucus manageable

What makes mucus such a sticky issue in people with cystic fibrosis, and what are Cystic Fibrosis Trust-funded researchers doing to tackle it?

At the European Cystic Fibrosis Society’s Basic Science Conference last month Dr Vinciane Saint-Criq, an early career researcher working on the Trust’s ‘INOVCF’ Strategic Research Centre led by Dr Mike Gray in Newcastle, was awarded a prize for her research looking for new ways to make mucus easier to clear. But why is mucus in people with CF so different than in people without the condition, and what can we do to make it less dangerous and more manageable?

A sticky issue

One of the things we know about the lungs of people with CF is that they get clogged with thick, sticky mucus. As well as it being much thicker than in healthy lungs, the pH (the acid-alkali balance) of the mucus is also different – in CF lungs the mucus is more acidic. Both the thickness and acidity mean that it’s harder to keep the surface of the lungs clean and bug free.

What normally happens in healthy lungs is that the mucus is swept away, taking with it any bugs that enter the lungs, and the normal pH of the mucus helps to kill off bugs within it. In CF, as the mucus is thicker, it’s harder to sweep away, and the acidic conditions mean the bugs trapped in the mucus are not killed so easily. Drugs to treat CF have the effect of correcting the properties of the mucus and causing it to act normally.

Mending mucus

CFTR, the protein that is damaged in CF, sits at the surface of the cells that line the lungs. It acts as a gate to let chloride and bicarbonate through, which play a role in balancing the pH of the mucus and keeping it thin (watery) and easier to move.

As well as CFTR there are a number of other gates. Researchers think that some of these may also contribute to the pH of the mucus, but don’t know which. A treatment that works by targeting one of these other gates could potentially work for anyone with CF, rather than limiting it to those with a particular genotype or CFTR mutation.

New gateways for mucus clearance

In Dr Saint-Criq’s research, she is investigating whether any of the other ‘gates’ could help correct the properties of the mucus, and she has so far found two other gates apart from CFTR that might do the job, using a newly developed method for measuring mucus pH. Her award, which was supported by Bob Emmelkamp who is a long-time fundraiser for the Cystic Fibrosis Foundation, will enable her to attend the North American Cystic Fibrosis Conference this October.

Tonnes of Trust research

Janet Allen, Director of Strategic Innovation, who attended the conference said: “One in ten of the presenters received funding from the Trust, and the large number of presentations of Trust-funded research demonstrates the success of the Trust’s research strategy - both in terms of making progress towards a life unlimited and also in building a UK CF research community to lead the charge towards developing more effective treatments.”

Other attendees included researchers from the Netherlands, USA, Germany, Portugal and the UK, who are being funded by the Trust’s Strategic Research Centre programmes on gene editing, non-CFTR approaches to CF treatments (INOVCF) and CF-related diabetes.

Find out more about Dr Saint-Criq’s work at Soapbox Science in Newcastle on Saturday 16 June, where she and other female scientists will be taking to their soapboxes to wow the local community with cutting-edge science. Find out more about this free event here.

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