May 12, 2022
Watch Professor Simon Johnson's presentation recorded on Thursday 12th May 2022 about the rare lung disease, Lymphangioleiomyomatosis (LAM), at the Margaret Turner Warwick Centre (MTWC) for Fibrosing Lung Disease. Click here to watch the recording.
The MTWC lecture series is primarily aimed at researchers and healthcare professionals, which means that elements of each lecture are very technical. However, interested members of the public are welcome to watch the recording. Read on to find out more about what Professor Johnson talked about during his MTWC lecture.
Lung damage and repair in lymphangioleiomyomatosis (LAM)
Lymphangioleiomyomatosis, LAM for short, is a rare disease that slowly destroys normal lung tissue, leaving holes, or cysts within the lungs.
Although LAM and pulmonary fibrosis are different diseases, there are some similarities in the disease processes and techniques scientists use to study them. Understanding each individual disease has the potential to lead to better understanding and treatment of the other.
As the number of cysts increase, people with LAM become increasingly breathless and may suffer from collapsed lungs or pneumothorax. LAM is an incurable and life shortening disease that almost only affects women and is most often diagnosed before the age of 50. LAM is caused by a change in genes which are linked to a genetic disease called tuberous sclerosis. Although not all women with LAM have tuberous sclerosis, the link between the two diseases has allowed researchers to understand LAM and importantly the function of a group of proteins that control how cells grow, use nutrients and survive in stressful environments, known as the mTOR pathway.
In this talk I will discuss our work on how the lungs become damaged in LAM, with a focus on how the mTOR pathway activates lung damaging proteins, called proteases, to make lung cysts but also prevents the lung from repairing itself by reducing the efficiency of cells that can repair damaged lungs, a process called senescence. I will discuss how these processes can be targeted to treat LAM.
Interestingly, the mTOR pathway, the activation of proteases and how senescence can affect lung repair are common to both LAM and pulmonary fibrosis and have potentially important implications for each other.
Professor Johnson’s Biography
Simon Johnson is a Professor of Respiratory Medicine at the University of Nottingham. In addition, he is:
• Respiratory Theme Lead Nottingham NIHR Biomedical Research Centre
• Director National Centre for Lymphangioleiomyomatosis
• Co-director Rare Cystic Lung Disease Collaborative Network
• Member of the LAM Foundation scientific board
• Member of the Tuberous Sclerosis Association specialist advisory panel
His clinical interests are respiratory medicine, interstitial lung disease, Lymphangioleiomyomatosis and rare lung disease.
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