Professor Nick Morrell
BHF Chair of Cardiopulmonary Medicine
University of Cambridge
Professor Nick Morrell and his research team
are investigating why lung blood vessels narrow in pulmonary
hypertension, and how the narrowing might be prevented or
reversed.
Blood flows through the heart in a precise way. After
distributing oxygen throughout the body, blood returns to the right
side of the heart before being pumped to the lungs to become
oxygenated again. Oxygen-rich blood flows back to the left side of
the heart to start the cycle again.
Pulmonary hypertension is high blood
pressure in the arteries carrying blood to the lungs. It can
develop after long-term lung or heart disease, or from other
diseases that narrow the lung arteries.
Pulmonary hypertension causes breathlessness and significantly
shortens life expectancy. The severest forms lead to death
from heart failure within a few
years.
How are genes involved?
Pulmonary hypertension can run in families. It usually
happens due to an alteration in the gene for a specific protein,
called 'bone morphogenetic protein receptor type 2' (BMPR2).
This Cambridge team was the first to show how alterations in
BMPR2 cause faulty signals to be sent in cells lining the lung
blood vessels, leading to faulty control of these cells' growth and
survival.
They are now looking for ways to correct the underlying genetic
defects, including using drug therapies to restore normal function
of BMPR2, or replacing the faulty gene using gene therapy.
Why are lung blood vessels different?
Lung blood vessels behave differently from blood vessels in the
rest of the body. These different vessels are the only blood
vessels involved in pulmonary hypertension.
Alterations in BMPR2 only seem to have effects on lung
arteries. Studying this gene is fundamental to discovering how
pulmonary hypertension develops, and why lung circulation is
unique.
Professor Morrell's work suggests BMPR2 is also involved in
non-inherited forms of the disease. His studies will help us to
understand the different forms of pulmonary hypertension.