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All You Need to Know About Adrenoleukodystrophy

Myelin

The movie, Lorenzo’s Oil, is about a boy who has a disease called adrenoleukodystrophy (ADL).This disease is inherited through the female parent. Since it is inherited from the mother, families that have a child suffering from ADL often have much tension and pressure on the mother. Mothers often feel at fault for their children’s disease when there is nothing they could have done to prevent it.

ADL is a very rapidly progressing disease. Symptoms start between the ages of 4 and 10. Symptoms begin with a fading of motor skills and become more severe till the infected person dies. Between the fading of the motor skills and death victims become blind, deaf, and suffer from seizures. Victims lose muscle tone and control to the point where they are unable to swallow.

The disease strips away the myelin from the nerves. Myelin is a protective casing around nerves much like the plastic around electric wires. Without myelin the nerve signals are greatly slow and quite often not received. It occurs when there is a mutation in the Xq28 marker on the X chromosome.

The Odone family managed to find a treatment to slow down the symptoms and even get them to come to a halt through perseverance. They joined a group where they gained much knowledge on the disease. From there they spent countless hours in the library researching the disease. Michaela Odone, Lorenzo’s mother, stumbled upon a rat study by chance. In it certain levels of fats were reduced by introducing other fats. She felt that this could be applied to Lorenzo’s case to stop the disease. The family funded symposiums were scientists met together and exchanged ideas. Eventually, the Odones found a company that was willing to create an oil that they believe could heal ADL patients. This treatment was successful in stopping any further deterioration of the body and got fat levels back to normal. The treatment was called Lorenzo’s Oil.

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The oil is a mixture of four parts glycerol trifoliate and one part glycerol trierucate. They are forms of oleic and erucic acid. The oil works by normalizing saturated very long chian fatty acids (VLCFA) by shutting off the biosynthesis of saturated VLCFAs.

Due to the Odone family’s efforts the public knows much more about this disease now than ten years ago. As a matter of fact ten years ago this disease was just being discovered and was unknown to the public. Scientists have discovered that Lorenzo’s oil greatly helps battle against ALD. The increasing amount of information that is being discovered is greatly due to the Myelin Project.

After the release of the movie, the Odone family started the Myelin Project. It raises money to fund the research of a cure for ADL. Unfortunately, on June 10, 2000 Michaela Odone died of lung cancer. On May 30, 2008, one day after his birthday, Lorenzo Odone lost his fight against ALD. His father, brother, and sister are still alive.

Works Cited

Moser, Hugh, Ann Moser, Steven J Steinberg, & Gerald V Raymond. “X-Linked Adrenoleukodystrophy.” GeneReviews. 27 July 2006. GeneTests. 6 Feb 2009

National Institutes of Health. NINDS Adrenoleukodystrophy Information Page.” National Institute of Neurological Disorders and Stroke. 18 March 2009. NINDS. 2 Jun 2009 .