The skeleton key.

Fibrodysplasia Ossificans Progressiva (FOP) is a rare inherited disease that turns muscle into bone. It cannot be halted by surgery or any other method. Michael Zasloff, molecular biologist and paediatrician (Children's Hospital of Philadelphia), who witnesses the terrible effects of this disease in some of his child patients, is engaged in research into this condition that has so far evaded all attempts at treatment. He and his colleague Fred Kaplan, an orthopaedic surgeon confirmed that FOP is an hereditory disease and found that the gene for bone morphogenetic protein (BMP) was at fault. This gene did not switch off, thereby causing the development of FOP. BMP is carried in the lymphocytes. It had been noticed that FOP flares-ups occured after injury, and the reason was found to be that lymphocytes, responding to tissue damage, carried BMP to the site, where it killed healthy muscle tissue, turning it to bone. Further research led to the discovery that mice have a protein that controls the amount of skeleton made. Could FOP patients be treated with this protein? It would not cure the condition but could prevent it worsening. In the course of their investigations Zasloff and Kaplan studied the shark which has cartilege instead of a bony skeleton and they found that it was restricted blood flow which prevented cartilege from turning into bone. The shark carries squalamine, a substance which blocks the formation of blood vessels. Could the disease be controlled by selective control of blood vessel growth? It is anticipated that at least another two years work is necessary before it is known whether a drug can be developed.

A documentary about a rare hereditory disease called Fibrodysplasia Ossificans Progressiva that turns muscle into bone. The programme follows the research of two American researchers at the Children's Hospital of Philadelphia. In this respect they use both mice and sharks for experiments to find out how to control the disease that is at the moment both incurable and uncontrolable.

UK : BBC-TV, 1999.

1 DVD (50 min.) : sound, color, PAL.

October Films

October Films