Dies Lorenzo Odone, the ALD patient subject of “Lorenzo’s Oil” movie and Phil Collins’ “Lorenzo” song
“IT IS WITH GREAT SADNESS THAT WE ANNOUNCE THAT LORENZO ODONE PASSED AWAY MAY 30, 2008, IN HIS SLEEP AT HOME IN FAIRFAX, VA. WITH BOTH HIS FATHER AUGUSTO AND HIS LIFE LONG FRIEND OMOURI HASSANE AT HIS SIDE” ( The Myeline Project website )
Lorenzo, a patient who suffered of Adrenoleukodistrophy (ALD) since the age of 5 years, was the subject of “Lorenzo’s Oil” movie and the Phil Collins’song “Lorenzo”. According to AP, he died one day after his 30th birthday.
ALD, the disease Lorenzo suffered, is a peroxisomal disease in which the beta-oxidation of Very Long Chain Fatty Acids (VLCFA) is affected. Dietetic limitations of VLCFA has not proved to be effective in decreasing the levels of VLFC in affected patients, so endogen production is the most important source of these acids in our body and as is usual in metabolism, an equilibrium exist between the production and the degradation of these compounds.
While the beta oxidation of medium and long chain fatty acids occurs in mitochondria, the oxidation of VLCFA is initiated in the peroxisomes. In ALD there is lack of a functional ABC membrane protein, apparently necessary for the uptake of VLCFA by the peroxisomes. As a consequence, VLCFA, like hexacosanoic acid (C26:0), accumulate and damage the myelin affecting mainly the brain and the adrenal glands.
Trying to find a cure for his son, Augusto and Michaela Odone (none of them related to the medical profession), studied related Biochemistry topics by themselves.
During this process, A. Odone read a paper describing that the same microsomal system that participates in the elongation of saturated fatty acids to form VLCFA, also elongates erucic acid (22:1 n-9) to form nervonic acid (24:1 n-9).
This information gave him the idea of using the biochemical mechanism of competition, that he had learned previously, for decreasing the endogenous formation of VLCFA by administrating erucic acid to his son.
The Odones developed an special combination of fats (a mixture of 4:1 glyceryl trioleate –glyceryl trierucate ) that helped his son to fight the disease. (Odone A, Odone M. Lorenzo’s oil: a new treatment for adrenoleukodystrophy. J Pediatr Neurosci. 1989; 5:55-61). The levels of VLCAF in Lorenzo’s blood began to decrease a few weeks after the “oil” was administered to him.
Lorenzo did not die during the usual average of two years after the diagnosis of ALD. In fact, he lived until yesterday, more than 20 years later than expected by his doctors.
The Odones also founded “The Myelin Project”, a foundation devoted to funding research to find a cure for demyelinizating diseases.
Nowadays Lorenzo’s Oil is used in the treatment of some patients suffering of ALD. FDA considers it an experimental drug yet.
More information about Lorenzo and ADL can be found in the Biochemistry at the movies page and the links that appears in it.