Lack of Amino Acid Spurs Deadly Sickle Cell Complication

laboratory microscope lens in a labA deficiency of the amino acid arginine may contribute to the development of
pulmonary hypertension — a potentially deadly complication — in people with
sickle cell anemia, a new study suggests.

A process called hemolysis, in which red blood cells rupture, is what causes
the arginine deficiency, researchers said.

“Within the red blood cell, there is a high concentration of an
arginine-consuming enzyme called arginase,” said the study’s lead author, Dr.
Claudia Morris, the director of research for the pediatric emergency fellowship
at Children’s Hospital and Research Center in Oakland, Calif.

When arginase is released during hemolysis, explained Morris, that “triggers
a cascade of events that lead to pulmonary hypertension.”

The study findings appear in the July 6 issue of the Journal of the
American Medical Association

Sickle cell anemia is a disease that causes red blood cells to become
sickle-shaped. This abnormal shape makes blood clots more likely, and when a
blood clot blocks a blood vessel it can cause extreme pain, according to the
National Institutes of Health (NIH). These damaged blood cells are also more
likely to break down prematurely in a process known as hemolysis. Sickle cell
disease strikes African-Americans more frequently than other racial groups.