Dr Zlatko Kopecki: ‘Flightless’ skin barrier protein may be key to autoimmune blistering disease

Autoimmune diseases

18 Jul 2019

In 10 words, what are you currently working on?

Understanding the role of skin barrier in autoimmune blistering disease

What have you discovered in this area so far?

We have contributed to understanding the role of an actin remodelling protein called ‘Flightless’ in skin blistering by describing the first mechanism leading to the resolution of blistering and inflammation in an autoimmune blistering disease, called epidermolysis bullosa acquisita (EBA). Flightless protein is increased in the skin of patients with blistered skin, promotes skin blistering and delays skin barrier reestablishment following blistering. Reducing the levels of Flightless protein in blistered skin leads to improved healing of blisters. We have also described the role of this protein during skin development, and recovery of the intact skin barrier post skin blistering. Read more here and here.

Will this work help with preventing or managing autoimmune blistering disease – or both?

These studies are preclinical investigations that have identified novel targets for potential therapeutic strategies aimed at both reducing skin blistering and improving healing of blistered skin. Long term, these studies may lead to therapies which will help in managing symptoms associated with skin blistering in autoimmune blistering diseases like epidermolysis bullosa acquisita. Reducing the levels of Flightless protein in skin of patients is anticipated to reduce the extent of skin blistering and improve blister healing.

What aspect of this research interests you the most?

The most interesting aspect of this research for me is understanding how we can use our knowledge of the skin barrier to develop and apply wound healing therapies aimed at decreasing the clinical symptoms associated with autoimmune skin blistering disease.

Have we underestimated the role of the skin barrier in autoimmunity?

Yes, to a degree I think the role of skin barrier in autoimmunity is often regarded as a secondary consequence of skin blistering. However, therapies focused on restoring the integrity of skin barrier hold great promise for addressing the clinical symptoms in autoimmune skin blistering disease.

How far is this work from impacting patient care?

Our preclinical studies are still 10 years away from having a potential impact in patient care as we first need to conduct human clinical trials. The interest in developing therapies aimed at restoring the skin barrier is high and hopefully we will see some of these therapies progress to patient care in the not too distant future.

What’s been your biggest research hurdle?

One of the constant research challenges is ensuring we are using the best and latest disease models in preclinical animal studies as we are constantly finding out new information. We have a strong focus on ensuring our research is clinically relevant to human disease and driven by patient needs. A second challenge faced by many researchers is obtaining research funding to translate preclinical research findings into human clinical trials.

What’s your Holy Grail – the one thing you’d like to achieve in your research career?

I dream of a day when impaired wound healing and associated pain and infection of blisters will not be a problem for patients with severe skin blistering. Great progress has been made in the last ten years and there are currently many clinical trials being undertaken to translate research findings into clinical practice. I hope to one day be involved in development of a therapy that will be used in the clinic to help patients live a more comfortable life.

Who has influenced you most in work or life?

Two people who have influenced my work the most are Professor Allison Cowin (University of South Australia) and Professor Ralf Ludwig (University of Lubeck, Germany). Professor Cowin is a renowned expert in animal models of wound healing, with whom I work closely and still learn new things from every day. She has helped me understand the importance of research translation and therapeutic development. Professor Ludwig is a leading dermatology expert in autoimmune skin blistering diseases, especially epidermolysis bullosa acquisita, with whom I have worked closely. His mechanistic research and clinical understanding have inspired me to develop a strong interest in autoimmune skin blistering diseases.

There’s an app for that. What’s new on your phone?

Last app I downloaded is called Wound Pro, it is an easy to use app for wound measurement, analysis, assessment and management used by different wound specialists both in clinics and in research.

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