Obituary: Lorenzo Odone
June 5, 2008 | Source: New Scientist news service
Lorenzo Odone, who died last Friday aged 30, was the subject of the film Lorenzo’s Oil, which made him the most famous person to suffer from adrenoleukodystrophy (ALD).
ALD is an inherited disease in which the myelin sheaths around neurons are destroyed. Without myelin, the neurons cannot conduct signals properly, causing brain damage and seizures. Children with ALD generally die within a few years of being diagnosed. The myelin damage is thought to be linked to high levels of very-long-chain fatty acids.
Reasoning that they could combat the disease by reducing the levels of very-long-chain fatty acids, they developed an oil derived from rapeseed and olive oil. When they gave the oil to Lorenzo, it reduced the levels of very-long-chain fatty acids in his blood, by blocking the enzymes that make them.
The oil does protect boys if it is given when they are still young and show no symptoms, though it does not help people whose myelin is already damaged.
The Myelin Project aims to fund and drive research into myelin diseases.