What is Cystic Fibrosis What is SCID4/9/2024 The researchers also showed that adding existing medicines, licenced for other conditions, improved the alkalinity of the mucus layer. They found that both TMEM16A and SLC26A4 proteins are definitely involved in balancing the right acid/alkali levels. (Like the clever tech they use on the screens, in the post-match analysis on TV sports broadcasts, to track a particular player’s role in the action!). Studying what each protein did on its own meant the researchers could track its contribution to the team dynamics. They used genetic engineering to inactivate or ‘switch off’ each of these proteins in turn and looked at what happened to the acidity levels of mucus. (Like the CF protein – the full names of these proteins are so long, they are only ever referred to as their abbreviations!). The most common (in 70 of people with CF) is a three-base deletion in the DNA sequence, causing a single amino acid to be missing from the protein product. In particular, they looked at two other proteins that work on the surface of the lungs called ‘SLC26A4’ and ‘TMEM16A’. Interesting facts about cystic fibrosis More than 1,000 different variations (alleles) of the CFTR gene have been identified in people with CF. People with Class 1 CF mutations don’t make any CF protein, and they are not able to benefit from medicines such as Kaftrio. The researchers used cells collected from the noses of people with CF who had rare ‘Class 1’ mutations and studied them in the lab. ![]() Studying cells from people with rare CF mutations This work was done in collaboration with researchers from Professor Jeff Beekman’s lab at University Medical Centre Utrecht in the Netherlands, who were also co-investigators in the same SRC. Newcastle-based researchers Dr Livia Delpiano and Dr Mike Gray, working on one of the Trust’s Strategic Research Centres (SRCs), looked at what happens to the acid-alkali balance of mucus when the CF protein isn’t made and whether they could improve the health of lung cells in the lab. Understanding the complex ‘team dynamics’ of how these proteins work together could lead to new ways to treat CF, particularly for people with Class 1 type of CF mutations that are not able to benefit from CFTR modulator medicines such as Kaftrio. Just as players in a football or rugby team work together during a match, each having their own specialties but equally able to cover for each other at different times, the same is true for the proteins on the surface of the lungs. It works very closely with other proteins on the surface of the lungs, too. However, the CF protein doesn’t do this on its own. The CF protein is involved in keeping the lungs healthy and in balancing the acidity of the mucus. Correcting the mucus back to an alkaline solution (alkaline pH) could be an effective way to treat CF. In people with CF, the mucus becomes too acidic in turn, this increases its stickiness and prevents bugs trapped in the mucus from being removed or killed. ![]() One way to do this is by ensuring the correct acid-alkali balance (correct pH) of the mucus that lines the surface of the lungs. CF BioResource project – understanding your cystic fibrosisĪn important part of keeping the lungs healthy is to keep them well-hydrated and clear of infections.Easy read information on cystic fibrosis.What are the causes of cystic fibrosis?.
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