This emphasises the need to use the treatment efficiently. Our initial studies focused on how the enzyme interacts with isolated Gauchers cells grown in the laboratory. The intercellular half life of the enzyme is up to 60 hours. This means that after the enzyme enters the Gauchers cell, approximately half is excreted after two and a half days. Ceredase and Cerezyme bind to the mannose receptors on Gauchers cells but other receptors may be involved.
Receptors are like letter boxes through which the enzyme can pass .
Dr Mistry then described how Ceredase and Cerezyme were modified to follow their itinerary through the body after infusion. The patients were continuously scanned to follow where and how the enzyme was reaching parts of the body affected by Gauchers disease. The highest amount of enzyme was taken up by the liver, followed by the spleen and the bone marrow reflecting exactly the distribution of fat-engorged Gauchers cells in the body. However there was little uptake in the lungs at the extremely low doses given to these patients. More Ceredase and Cerezyme was taken up in the livers of patients who had previously had splenectomy.