http://www.nrlc.org/news_and_views/April10/lorenzosoil.jpg. Biography Full Name: Lorenzo Odone Born: 29th May 1978 Passed Away: 30th May 2008 Age: 30 years of age Location: Virginia, Washington Country: The United States Father: Augusto Mother: Michaela.
Full Name: Lorenzo Odone
Born: 29th May 1978
Passed Away: 30th May 2008
Age: 30 years of age
Location: Virginia, Washington
Country: The United States
Lorenzo was an intelligent and happy young boy….until
Lorenzo’s behaviour drastically changed for the worse.
They then ran various tests to find out what was wrong…
Lorenzo had the neurological disease Adrenoleukodystrophy (ALD).
There is no transporter protein to transport an enzyme to breakdown long chain saturated fats therefore:
Certain cells are made by the saturated fats, however without the enzyme to destroy extra fat, the excess it builds up
Lorenzo was given 2 years to live.
The Desase is still progressing however. They observed hemianopia, with transient horizontal nystagmus. The pupillary light reflexes were still intact, as yet, there was no optic atrophy.
In response, they head to the ALD Family Conference in July.
Others were experiencing the same results as the Odone’s were.
Through studying, Augusto described Lorenzo\'s condition through the use of a sink:
Tap on the right represents very long chain saturated fats. Now, also produced by the body or manufactured by the body, are these very same fats (biosynthesis). It shouldn\'t be problem since an enzyme breaks down the excess, however in Lorenzo’s case the enzyme is not present. In the case of the sink, the drain is blocked.
While at the library, Michaela had stumbled upon an article about fatty acid manipulation
In a polish study, when rats were deprived of a specific fat in their diet, body cells compensated by overproducing it.
So they hypothesized that they can stop the body from producing one kind of fat:
By loading it with another:
They discussed how the clinical trial was not working.
The case study was also brought up which shed a new a light on the disease.
And with the combined knowledge of researchers from all over the world…
If it is in its pure form, it becomes hazardous however if it is in its triglyceride form then it is edible.
From olive oil
Companies were being stubborn
Also, Lorenzo’s condition worsened to a point were he needed spit suctioned out of his mouth.
And the Odone’s were sent an extra bottle.
Is it really ethical of the Odones to put untested substances into Lorenzo?
Blood was drawn for a baseline
Lorenzo was given the oleic oil through food
A small percent drop of C24 and C26 was found in Lorenzo’s blood work.
Not soon after the call did Lorenzo suffer from paroxysm (saliva gets caught in the trachea.)
50% drop in both C24 and C26 fatty acids.
They were being quite stubborn about accepting the Odone’s research.
The decrease in C24 and C26 fatty acids were barely noticeable..
And, as time continued, Lorenzo’s condition continually becomes worse.
Each paper clip equaled 2 carbon atoms. These would connect to other carbon atoms in chains, such as C2-C4-C6. These chains where trying to make monosaturated fats. One chain was considered the "good guys" while the other chain was considered to be the "bad guys". The father was the "good guy enzyme" and took a carbon and put it on his chain. Deidra however was the " bad guy enzyme" and put a carbon on her chain. This was done at the same rate. However, what confused him was why when the faster the “good guys” went, the slower the “bad guys” went, if they were both separate enzymes?
The answer that had come to him was that they were both the same enzyme.
So if they keep the enzyme busy making monounsaturated fats, it stops the enzyme from elongating the chain.
This Process is known as Competitive Inhibition.
It explains why oleic oil was only partly successful, since it was too low down the chain. Therefore, by adding erucic acid, it creates a second barrier and blocks the “bad guys” further up the chain.
Just like the oleic acid, Dr. Nikolai was hesitant about using Erucic acid upon Lorenzo since it causes heart problems on rats.
Erucic acid however has to have its C24 and C26 fats removed and it is difficult to have synthesized.
A British chemist known as Don Suddaby working for Croda International decided to take on the task that the Odone’s had set.
Mr. Suddaby had managed to synthesize the erucic acid and had it delivered to the Odone’s.
Deidra was used a test “rat” for the erucic acid. There were no harmful after effects which therefore meant it could be given to Lorenzo.
Lorenzo’s blood sample had come back with C24 and c25 reading normal.
Lorenzo continued improving, even to point where he was able to swallow his own saliva.
A sample was given to Wendy, and the same results were received with her child. This sparked a potential of a trial to be created.
With at first being able to communicate with his eyes…
and then even being able to move a finger.
Meanwhile Lorenzo made it past the age of 20. He was able to move his head from side to side. He had also recovered his eyesight and had begun to vocalize simple sounds. He was learning to communicate by means of a computer. However, on May 30, 2008 (aged 30), he died from aspiration pneumonia.