LORENZO’S OIL INTRODUCTION
March 5, 2012
Lorenzo’s Oil, a film released in 1992, is a somber drama about parents, Augusto Odone (Nick Nolte) and Michaela Odone (Susan Sarandon), in their desperate fight to save the life of their son, Lorenzo. In the movie, which is set from 1984-1987, Lorenzo Odone is initially portrayed as a normal, happy, healthy child whose health suddenly declines. Upon learning of Lorenzo’s dire condition, called adrenoleukodystrophy, the Odones set out on a mission to find a cure for ALD and save the life of their son. Both Augusto and Michaela are very learned individuals, and rather quickly and in exhaustive fashion, they train themselves in biology and biochemistry in order to create an effective treatment for their son. In their fight, they challenge and push the medical establishment to its limits. No doubt, their passion and emotion do make a difference for their son and other families afflicted with ALD. This sympathetic portrayal of despair, agony, victory and defeat easily becomes an indelible memory for Human Biology students.
The movie did attract a good deal of acclaim for the courage of its content. In 1996, Phil Collins recorded a song as a tribute to Lorenzo, naming it "Lorenzo." The lyrics for this song were written by Lorenzo and his mother, Michaela. It was released on his "Dance Into the Light" album.
This week as we watch the movie, you should do some research on adrenoleukodystrophy or ALD, for short. ALD affects the nervous system’s ability to function due to the deterioration of the myelin sheaths (a white, lipid sheath surrounding neurons). Without viable neurons, the brain cannot effectively send messages to effector organs.
To further explain ALD, take a look at the word origin for adrenoleukodystrophy. "Leuko" means white (the myelin sheath that covers neurons gives them a white appearance). "Dystrophy" means "abnormal development." So, ALD is literally the abnormal development of the white, myelin sheaths surrounding neurons. There are many forms of ALD, each varying in severity. The type of ALD seen inLorenzo’s Oil is one of the worst. Generally, boys afflicted with ALD begin showing symptoms between the ages of 5 and 12 and death is imminent within a couple of years. The symptoms (dementia, loss of sight, hearing, speech, and ability to walk-ambulation) are believed to be due to solubilization and removal of the myelin sheath surrounding neurons because of a build up of very long chain, saturated fatty acids (VLCSFAs) in the body (Moser, 1997). Nerve cells cannot conduct action potentials as effectively without the myelin sheath, and as deterioration worsens, neurons will not conduct action potentials at all. From your introductory understanding of the importance of action potentials, you know that the inability to conduct action potentials is a deadly problem. Multiple sclerosis, using a different mechanism, will also remove myelin, leading to paralysis as well (Stith,
The following websitesshould helpboth in your comprehension of the biology/biochemistry presented in the movie andin answering the following set of questions, which are due on Friday, March 23, 2012.
Remember, these questions will be graded as a take-home test. This is PLEDGED work. We will work on these questions one day in class, and at this time, you may ask me questions about the assignment. You may NOT turn in the same answers as another student. Your answers must be your own. If you do not understand or need help to complete these questions, you should set up a time to work with me in tutorial!
Answers to the question set must be typed!
References:
website of Bradley J. Stith, Biology Professor, University of Colorado
LORENZO’S OIL MOVIE QUESTIONS
Questions are due on Friday, March 23, 2012
1. In the movie, Augusto made a breakthrough discovery about the enzyme that was producing the “very long chain saturated fatty acids or VLCSFAs”. What was it? Explain the concept of competitive inhibition as it relates to enzymatic activity?
2. What were the two major paradoxes in the movie? Explain them and how models and explanations were developed for both.
3. Does Lorenzo have one or two copies of the gene that causes ALD? Does his mother or father have one or two copies? For each person, note whether the copy is defective (recessive) or normal (dominant). Write the genotype for each person.
4. True or False: Most inherited diseases like ALD or cystic fibrosis are recessive diseases. What does the phrase "recessive disease" mean?
5. If most inherited diseases are due to the recessive allele, and many patients die before they reproduce, why does the recessive allele remain in the population?
6. Why are boys more likely to have a sex-linked inherited (recessive) disease than a girl? Use a Punnett square. Show your work.
7. What if Lorenzo was able to have children: what would be the odds of having a child with ALD if Lorenzo married a carrier (calculate for a female child and then a male child) or a non-carrier individual? Use Punnett squares. Show your work.
8. Go through the genetics of a non-sex linked recessive disease; that is, show the genotype of parents where there are two copies of the gene involved in an inherited recessive disease. Must both parents be heterozygotes or carriers for a child to be stricken? Will both girls and boys be affected with equal probability? What is the probability of two carriers producing a normal child? Use Punnett squares to explain your answers. Show your work.
9. With ALD (prior to the development of Lorenzo’s Oil), why was it not possible for a female to have the genotype of homozygous recessive (from figures, xx)? Why, in some other sex-linked or autosomal inherited diseases, is it possible to have this genotype (xx or aa)?
10. Is it appropriate for medical researchers to take the kind of chances with their patients that the Odones took with Lorenozo? Lorenzo's Oil is not without side effects. The oil reduces platelet count (thromboytopaenia)- what medical problems would this produce?
11. What do you think about drug companies that refuse to study diseases like ALD? Their reason is that there are few patients and no large profit in developing an ALD drug. Is this an acceptable reason? The drug company may not stay in business if it does fight ALD. Should government develop new regulations that state that a percentage of profits be used to develop drugs for orphan diseases?
12. If you were a carrier for ALD, would you have a child? Would it make a difference if you knew it was a girl or a boy? Should you be prevented from having a child by law? If the inherited disease was not sex-linked and both parents were carriers (you should have already worked out the relative % of normal vs. carrier vs. affected offspring), would you have a child?
13. At the end of the movie, Augusto begins tackling his next challenge – stem cells. Do some research on stem cells and explain your views on this type of research. Do you think that the stem cell research is ethical? If so, to what extent? If not, why not? Do you agree or disagree with the government’s current stance on the use of stem cell research?
Please be sure to cite any independent sources that you use to answer these questions.
Like we have discussed in class, forgetting to cite your sources or not properly citing your sources will be considered as a potential honor offense. You must take care of business on this front!
I am happy to help you understand these questions . . . so come see me in tutorial. I am a good resource, and I want all of you to make an A on this assignment!