Hacker Cinema
Lorenzo's Oil
1992 · Film · 135 min
Directed by George Miller
Lorenzo Odone, a Virginia 5-year-old, develops a degenerative nerve disease so rare that nobody is working on a cure, so his parents decide to immerse themselves in research and tackle the problem themselves.
3 positive5 neutralfrom 8 commenters
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8 comments on Hacker News
Yes I can explain and it's not big deal:
Morals are tenets of right and wrong. (Grace)
Ethics is conduct judged under moral principle. (Law)
Morals are truisms of the human disposition towards goodness.
Ethics guide conduct towards just ends where situations may be morally uncertain or ambiguous.
Morals are what we instinctively know about goodness, while ethics are the rules by which we collectively govern conduct to goodness.
— Lorenzo's Oil (1992) https://m.imdb.com/title/tt0104756/
Augusto Odone: When we first went to the Comoros, what did we do? We got to know the country, right?
Michaela Odone: Yes
Augusto Odone: We studied, we got to know the language, resources, its law. We studied, right? We should threat Lorenzo's illness like another country.
Michaela Odone: I don't quite see the analogy.
Augusto Odone: All right, all right. ALD has many dimensions, right?
Michaela Odone: Yes
Augusto Odone: So, in order to understand it, we need to command genetics, biochemistry, microbiology, neurology, ology-ology.
Michaela Odone: Augusto, we don't have time to go to medical school.
Augusto Odone: Michaela, the doctors are in the dark. They're groping in the dark. The've got Lorenzo on a turvy-topsy diet. And that bloody immunosuppression is brutal and useless. Michaela, we should not have consigned him blindly into their hands. He should not suffer by our ignorance. We take responsibility. So... we read a little. And we go out and inform ourselves.
Michaela Odone: But... to miss time with him while he can still speak to us...
Augusto Odone: Yes, I know, I know. But he expects it of us.
> What else are fatty acids used for?
Energy storage, hormone synthesis.
> What are the metabolic pathways involved and how do different fatty acids impact our cells?
Triglycerides are used as a substrate for energy production by mitochondria. Short-chain fatty acids with chain lengths < 22 can readily enter mitochondria and be turned into energy. Long-chain fatty acids cannot enter mitochondria; but they can be shortened by peroxisomes, another cellular organelles, to more feasible lengths which then can enter mitochondria. This whole "fatty" metabolic pathway is called beta-oxidation.
Both glucose and lipids are transformed into substance called Acetyl-CoA which is then used by mitochondria to produce ATP. Acetyl-CoA is a common denominator of our energy production pathways.
We can also imagine a software pattern when there is a main implementation (mitochondria working internally from Acetyl-CoA) and two adapters for glucose -> Acetyl-CoA (glycolysis) and lipids -> Acetyl-CoA (beta-oxidation) to simplify the whole thing.
Trans fats cannot be processed neither by mitochondria nor peroxisomes because they belong to a different class of fatty acids. This causes their accumulation inside the cell and a gradual development of a metabolic dysfunction because mitochondria cannot produce enough ATP (energy) from trans fats. If the accumulation is large enough it starts to block other metabolic pathways including glucose pathway. The mitochondrial "hunger" increases even more. Because glucose cannot enter mitochondria anymore, its level is rising leading to a condition known as insulin resistance. What happens next? A person may develop quite a few secondary conditions such as T2DM, hyperlipidemia, neurological and cognitive impairment, various cardiovascular and autoimmune diseases.
> Can you cite sources for your claims? Research is a passion of mine and I would love to read some literature on the topic if you have any to share.
I would gladly do that, but the list would be enormous. Materials on Krebs cycle are probably the best starting points. The next important milestone is to read information about Beriberi and Pellagra diseases because those conditions are caused by mitochondrial dysfunction which in turn is caused by nutritional deficiencies. The next thing to study is that metabolic dysfunction can be acquired not only from nutritional deficiencies but from a condition known as oxidative stress, which in turn can be caused by viruses, bacteria, poisons and so on. It can be acquired from the pathological excess of sugar during 10-20 years. Metabolic dysfunction can also be caused by genetics because some enzymes in metabolic pathways do not work as they should. To get a grasp of that, you can watch "Lorenzo's Oil" movie to see how it happens and what kind of sufferings a person may have.
As you can see, the amount of materials is quite enormous and hardly fits into a web-page-with-links format.
https://en.wikipedia.org/wiki/Lorenzo%27s_Oil
> Subsequent research with Lorenzo's oil has not clearly proven its long-term effectiveness in treating ALD after its onset.[8] The actual subject of the film, Lorenzo Odone, died of pneumonia in May 2008 at the age of 30, having lived two decades longer than originally predicted by doctors
A really good movie about myelin-degeneration (a disease called Adrenoleukodystrophy) is Lorenzo's Oil.
I know Morricone's name due to the beginning of Metallica's S&M album with the San Francisco Symphony:
https://www.youtube.com/watch?v=kKypYslGEA8
Although it was enough to get me to pick up "Yo-yo Ma plays Ennio Morricone" that I also recommend:
https://www.youtube.com/watch?v=KQCGvfBc-oI&list=PL2D68C2EE484F4AE2
Despite the huge list of movies he has composed for, the only one I might have seen is "Lorenzo's Oil", and I'm not sure if I actually saw it or just heard the name quite a bit due to living in Pittsburgh at the time.
https://en.wikipedia.org/wiki/List_of_compositions_by_Ennio_Morricone
all the best to the families. but this makes me furious at how research is done.
everyone pays high tuition and dont think about it. just being glad their families can afford. but you (everyone here probably) is fostering this. expensive pedigree universities are the ones that create such toxic environment for open research.
very similar to this was already documented in the "Lorenzo's oil" book/film. lets hope this time we learn the lesson. (yeah, high hopes of anyone here not favoring a Stanford student but anyway ...i have no idea how even start to break this vicious circle that promote inefficient research in that way... maybe killing patents will be the light?)
Reminds me of the Odone story as dramatize in the film "Lorenzo's Oil"
Reminds me of Lorenzo's Oil http://www.imdb.com/title/tt0104756/