Lorenzo`s Oil Science Movie Night Discussion

Transcription

Lorenzo`s Oil Science Movie Night Discussion
LORENZO’S OIL
SCIENCE MOVIE NIGHT
The Academic Support Center @ Daytona State College (Science 86 Page 1 of 23)
Presents:
The Academic Support Center @ Daytona State College (Science 86 Page 2 of 23)
Details:
Year: 1992
Rated: PG-13
Parental Rating: Cautionary; some scenes objectionable
Running Time: 135 min.
Released By: Universal
Awards:
Academy Award Nominee: Best Actress - Susan Sarandon
Academy Award Nominee: Best Original Screenplay – George Miller
Cast:
Nick Nolte: Augusto Odone
Susan Sarandon: Michaela Odone
Peter Ustinov: Professor Nikolais
Zack O'Malley Greenburg: Lorenzo
The Academic Support Center @ Daytona State College (Science 86 Page 3 of 23)
• Lorenzo’s Oil is based on the true story of a six year old
boy named Lorenzo Odone, who begins to exhibit the
symptoms of an extremely rare disease called
Adrenoleukodystrophy (ALD).
• When he was first diagnosed in 1984, ALD was still a
newly-discovered disease with no cure and extremely
limited available research.
• Lorenzo’s parents, Augusto and Michaela Odone, decide
to conduct their own research in a desperate attempt to
locate a cure for their son’s disease.
The Academic Support Center @ Daytona State College (Science 86 Page 4 of 23)
• an X chromosome-linked (sex-linked)
recessive disease
• passed from mothers to sons through
a defective X chromosome
Defective chromosome
Affected
Sex
chromosome
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• The defective chromosome inhibits the function of the specific
enzyme that breaks down very long chain saturated fatty acids
(VLCSFA), causing them to accumulate in the nervous system.
VLCSFA’s
= C24 & C26
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• accumulation of VLCSFA
destroys the myelin sheath that
insulates the axons of neurons
• demyelination inhibits the
transmission of nerve signals
The Academic Support Center @ Daytona State College (Science 86 Page 7 of 23)
The Academic Support Center @ Daytona State College (Science 86 Page 8 of 23)
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• What are some of the symptoms that Lorenzo
displayed, and how did they progress?
• loss of hearing
• loss of sight
• loss of balance
• loss of speech
• loss of muscle control
• inability to swallow
• Why did the symptoms and their progression lead doctors
to believe that Lorenzo had a degenerative disease that
affects the nervous system?
• accumulation of VLCSFA destroys the
myelin sheath that insulates neurons
• demyelination inhibits the
transmission of nerve signals
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• What treatment did Lorenzo first implement
to attempt to decrease the level of very long
chain saturated fatty acids in his body?
Dietary modification
• He decreased his nutritional intake
of saturated fats
• What effect did this have on
his VLCSFA levels?
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Augusto uses a diagram of a sink to represent
what is happening in Lorenzo’s body.
• In his diagram, what do the two
taps represent?
• The 1st one represents the long chain
saturated fatty acids that Lorenzo takes
in through his diet.
• The 2nd tap represents the VLCSFA’s
that Lorenzo’s body biosynthesizes.
• What does the drain represent?
It represents the enzyme that
metabolizes the VLCSFA’s.
The Academic Support Center @ Daytona State College (Science 86 Page 12 of 23)
• Why are Lorenzo’s VLCSFA levels increasing, despite the
fact that he has decreased his dietary intake of saturated
fats?
Biosynthesis & Homeostasis
• In addition to the very long chain
saturated fatty acids that are taken
in through diet, the body also
synthesizes its own VLCSFA’s.
• To compensate for the decrease in dietary intake of saturated fatty
acids, the body increases biosynthesis to maintain homeostasis.
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Michaela Odone discovers research on fatty acid manipulation that
provides a possible solution to Lorenzo’s high level of VLCSFA’s.
• What is the name of the substance that was first used
to manipulate Lorenzo’s fatty acid levels? Where does it
come from?
oleic acid (monounsaturated C18) =
Principle component of olive oil
The Academic Support Center @ Daytona State College (Science 86 Page 14 of 23)
• How does this substance help decrease
Lorenzo’s level of VLCSFA’s?
Increasing the intake of unsaturated fatty acids (oleic acid – C18)
causes the enzyme that synthesizes very long chain saturated fatty
acids to stop making them in excess. (has the opposite effect of
decreasing saturated fat levels)
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Oleic acid successfully decreases Lorenzo’s
level of VLCSFA’s for a period of time.
• Why did his VLCSFA level plateau after an
initial period of decrease?
Even though extra unsaturated fat is being added
to the diet, which decreases biosynthesis, the
body will still continue to synthesize a certain
amount of very long chain saturated fatty acid.
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Augusto Odone discovers another substance that
may provide a solution to the plateau that occurs in
Lorenzo’s VLCSFA level.
• What is the name of this substance, and where
does it come from?
erucic acid (monounsaturated C22) =
Principle component of rapeseed oil
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• What does Lorenzo’s oil consist of?
4 parts oleic acid
+ 1 part erucic acid
= Lorenzo’s Oil
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• How does Lorenzo’s oil stop the progression of ALD?
The same enzyme metabolizes both monounsaturated &
long-chain fatty acids.
Competitive Inhibition
• Erucic acid (monounsaturated C22) is long enough to compete with the very long chain
saturated fatty acids (C24 & C26) for the enzyme’s active site.
• This results in the production of more very long chain monounsaturated fatty acids and less of
the harmful saturated type.
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Unfortunately, Lorenzo’s oil can only slow down
or stop the progression of Adrenoleukodystrophy
for those who already exhibit symptoms.
• What other treatments for ALD were being
researched when the movie was produced?
• Myelin Regeneration
• Gene Therapy
• Stem Cell Research
• How would a cure for Adrenoleukodystrophy offer
hope to those with other neurological conditions?
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If treatment begins before symptoms develop, Lorenzo’s oil seems to
prevent the disease from occurring in many individuals with ALD.
• Since many males with ALD are now living past the
age of puberty, could this increase the likelihood that
females will contract Adrenoleukodystrophy?
• If so, how?
Defective chromosome
Affected
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• Should individuals resort to their own “unscientific” experiments
when proven research is unavailable?
• Is “good science” contrary to compassion?
• Is it ethical for human beings to suffer or sacrifice themselves
for scientific advancement?
• Is there a point where life is no longer worth living?
(What constitutes “life”?)
• Should scientists be more collaborative and less competitive?
The Academic Support Center @ Daytona State College (Science 86 Page 22 of 23)
Questions
Compiled by T. Lazauski – Peer Tutor
Edited by D. Leonard – Learning Specialist
The Academic Support Center @ Daytona State College
http://www.daytonastate.edu/asc/ascsciencehandouts.html
The Academic Support Center @ Daytona State College (Science 86 Page 23 of 23)