Restoring the Fizz Strategic Research Centre: Pharmacological repair of bicarbonate transport in cystic fibrosis

This Trust-funded Strategic Research Centre programme will be run by Dr Paola Vergani at University College London, and aims to better understand the flow of bicarbonate in different organs affected by CF. Researchers involved hope their studies could inform future treatment choices and help us understand more about CF outside of the lungs. 

Some people will be familiar with the chemical sodium bicarbonate, sometimes known as ‘bicarbonate of soda’. It can be used to help make cakes rise and is also used for cleaning things around the house. If you add water to a teaspoon of sodium bicarbonate in a bowl or a mug, it will fizz, and it’s the ‘bicarbonate’ part of the chemical that causes this.

Bicarbonate is an important chemical in the body too, it can help keep fluids at the right acid-alkali balance. It has a job in releasing mucus in the lungs and other secretions elsewhere, and maintaining their flow or ‘fluidity’. For bicarbonate to do its jobs in the body, it needs a functioning CFTR protein, that is damaged or missing in CF.

The CFTR protein is found in different parts of the body, including in the lungs, intestines, pancreas and liver. It acts as a gate allowing the movement or transport of two chemicals - chloride and bicarbonate - out of cells. Researchers have conducted many studies to understand how chloride transport is affected in cystic fibrosis, but they know less about how bicarbonate transport defects lead to the symptoms of CF.

The aim of this Strategic Research Centre (SRC) led by Dr Paola Vergani based at UCL in London is to understand in detail what happens to bicarbonate transport in CF. They will investigate it in different forms of CF (where people have rarer CF mutations) and in different tissues, including the intestine and liver.

They hope that their lab-based studies could lead to ideas about new ways to treat CF in the future. These could be better tailored to individuals based on the form of CF they have and the severity of different symptoms. They also hope their research could lead to improved treatments for some aspects of the disease.

This research is co-funded by the Trust and the Cystic Fibrosis Foundation in the United States.

Principal Investigator

Dr Paola Vergani, UCL, London

Co-investigators

Dr Marcel Bijvelds, Erasmus University Medical Centre, Rotterdam, The Netherlands

Professor Isabelle Sermet-Gaudelus, Institute Necker-Enfants Malades, Paris

Dr Guy Moss, UCL, London

Professor David Sheppard, University of Bristol