In Meet the Researcher, a series of interviews about inspirational academic research at St George’s, University of London, our people explain their work and its impact on society.

Professor Debbie Baines has worked at St George’s since 2000, when she was appointed as a tenure track scientist. Her main research focus is on the study of ion and solute transport across airway epithelium, and how hyperglycaemia may promote respiratory infection.
She was appointed Professor of Molecular Physiology in August 2013 and works within the Institute for Infection and Immunity.

Could you describe your most recent research activity?

I am helping to develop a gene editing treatment for cystic fibrosis. We are part of a Strategic Research Centre funded by the Cystic Fibrosis Trust looking at this, which includes the Francis Crick Institute, UCL, and the University of Cork.
Cystic fibrosis is caused by a genetic mutation in a specific gene known as CFTR. There are many consequences to this but one is the build up of thick sticky mucus in the lungs of patients. With gene editing we aim to precisely repair the mutation in the patient’s gene to correct the lung-related problems. The good thing about the airway epithelium is that it is relatively available to treatment– i.e. you can get at it. So one approach we are investigating is creating a medication which could be inhaled to correct the CFTR gene in the lung.
The other approach is where the patient’s own basal cells (the stem cells of the airway) are taken and gene edited in the laboratory; then put back into the patient’s lungs. But the technology for that is really complex and that is perhaps further away in the future.
Each of the institutions involved has a different role, and St George’s role in this is the responsibility for functional testing to find out whether the group’s strategies are proving to be successful or not. To do this we are working with in vitro systems of airways. Some of them are cell lines, some of them are cells that have been isolated from patients with the disease and grown so they act like miniature sections of the lung.

How does this link into your previous work?

I was looking at the role of diabetes in the airway with funding from MRC and Astra Zeneca and it links closely to that. Cystic fibrosis patients become diabetic later in life because of the disease and that makes their airway disease worse. I’m also investigating with my students whether diabetes affects the resident microbes in lungs – i.e., does it predispose you to infection? So one of my students is working on microbiome and the other is working on how the defence systems of the lungs might be compromised in diabetes.

How did you originally become interested in this area of research?

I did my PhD on sodium channels in nerve and muscle. Then a job came up investigating sodium channels in epithelia. I hadn’t worked in epithelia before, but I knew it was linked to cystic fibrosis. My best friend died at 21 from cystic fibrosis and so it had always been a bit of an interest with me. So it struck a chord, and I think it was a good fit.
Life expectancy of cystic fibrosis is now 38 – it has improved enormously in the last 20 years. That’s mostly through digestive enzyme replacement, antibiotic therapy and physiotherapy. It’s a multi-organ disease but there is much better management of the disease and earlier recognition of it. It’s a recessive hereditary disease. So there’s gene testing now. Few people know that one in 25 people are carriers of cystic fibrosis – but it depends on whether you meet another carrier as to whether your children might get the disease.

How does your research here fit into the wider picture of respiratory diseases?

There are a lot of similarities with COPD (chronic obstructive pulmonary disease) – both are to do with inflammation, balance of the lining fluid of the lungs and mucous which ultimately affects our ability to breathe properly. There is some evidence that if you smoke it might affect the cystic fibrosis gene product. In a normal airway this gene has a role: in a cystic fibrosis patient it has mutated so it can’t do its job. There is some evidence that smoke may also prevent it working properly.
Diabetes has a crossover with both as well. People with COPD and with cystic fibrosis often get diabetes. With COPD it’s related to obesity and reduced ability to exercise leading to type 2 diabetes; cystic fibrosis causes breakdown of the islets in the pancreas that make insulin so it’s more like a type 1 diabetes. But in both cases, lung function declines.
I have been lucky to be able to work closely with a very good clinical contact colleague here, Professor Emma Baker, who specialises in respiratory medicine, to be able to explore these crossovers.

What motivates you in your work?

Well, I love a problem -if there’s a knot in a piece of wool I have to untangle it! I like a challenge every day. Although I briefly worked in industry, I like the freedom that we get in academic science and that every day is different. It is nice to think that you might be able to find something that might change the management or the treatment of a condition. Coming up with drugs is really tricky; but managing people better from the knowledge that you’ve gained can help hugely.
George’s has always been an amazingly supportive place. I came here as a tenure track scientist and I’ve never felt the need to move on. I think our students also benefit from that supportive environment – they have more of a voice in how things are here.

What are your interests outside St Georges?

I’m a Trustee and Chair of Publications for the Physiological Society. It gives me an insight into how the charity sector and academic publishing works which is very informative, as well as insight into the present open access discussions that are dominating the sector. I’m also a committee member for the American Physiological Society. With both Societies, I am passionate about supporting the development of young scientists.
Outside of work I like sailing and skiing. I went across the Atlantic last year with the ARC fleet, which was amazing. I also like horse riding. I’m outdoorsy when I’m not in the lab!