Movie slumps are the worst. You know when you’re looking through the titles on your streaming services, but nothing sounds quite right? And as you continue to look with little success, you get increasingly frustrated until you decide not to watch anything at all. Okay, that’s a little dramatic but I’m sure it has happened to us all. One way to get out of this slump is to ask for recommendations. Instead of going for Netflix’s usual “you may like…” personalized list, I decided to ask a friend.
“Have you ever watched Benjamin Button?” he said. I hadn’t but I still wasn’t satisfied by this suggestion, but the polite thing to do was to smile and half-heartedly agree to watch it. I looked it up and saw that Brad Pitt was the star but not even that was enough to convince me to watch it. So, back in the slump I went.
The next week fate stepped in when a new lecturer was starting in my advanced bio-organic chemistry class. She began to discuss the investigation of novel therapeutic targets and to my surprise, a familiar movie cover appeared on her lecture slide. “The Curious Case of Benjamin Button,” with Brad Pitt sitting in a wheelchair, looking almost unrecognizable. What are the odds?! My professor then went on about the science behind the movie, but all I was thinking about was watching it as soon as I got home that day.
So, I did.
Of course, the movie doesn’t discuss any of the science I learned about in class, but it was still entertaining. We follow Benjamin, an orphan who is taken in by a woman who helps run a nursing home. Benjamin was “prematurely old” but was considered a miracle child, because even though he was a newborn, he looked like he was on his way to the grave. Throughout the years, he aged in reverse, getting younger and spryer as the years went on. Now I won’t spoil the ending for you because it is worth the watch, but I will let you in on the science.
From the screen to reality
Benjamin Button’s exact condition may be fictional, but the disease that inspired it is very real. It is known as progeria or more specifically Hutchinson-Gilford progeria syndrome. Progeria in Greek means prematurely old, and that’s exactly what the syndrome causes to its sufferers. Some of the symptoms include stiff joints, protruding eyes, aged and worn skin, hair loss, heart disease, among many other life-threatening health problems that young children shouldn’t be affected by. This extremely rare disease (affecting 1 in every 4 million births worldwide) is always fatal, with children living an average of 14 years before succumbing to atherosclerosis (or other heart related issues). This is the same disease that claimed Adalia Rose Williams, the young and vibrant YouTube star who spread awareness about the disease though documenting her life and sharing various tutorials.
Some people defy the odds of this disease, like Sammy Basso, an Italian microbiologist who lived until 28 years old. He dedicated his life to science and raising awareness about progeria, including researching gene-editing therapy to treat this disease.
Now we know what it is, but what causes it?
What causes progeria?
Progeria is a spontaneous genetic disease caused by a mutation in the LMNA gene. LMNA provides the instructions for making Lamin A protein, which is a major component to the scaffolding structure of our cells’ nuclei. The nucleus of the cell is what runs the cellular show. It is like the boss’ office that instructs all the other workers in the cellular factory to do their jobs. Without a properly functioning nucleus, our cells cannot live properly.
So, with a defective LMNA gene, abnormal Lamin A protein is being produced, causing cellular instability.
The devil is in the details
If we want to dive a bit deeper, the mutation of the LMNA gene causes problems in the maturation pathway of pre-Lamin A protein. This is a lot to digest but bear with me.
Protein synthesis is a complex process that occurs in many steps with several moving parts. What controls these moving parts is the gene. In the case of Lamin A synthesis, the main focus is its maturation.
Just like humans, proteins have to grow up. They start off as immature proteins that are long and contain a bunch of extra pieces. To process these “preemies”, different components of cellular machinery are called by the gene to do their job. These components are enzymes, and they remove any unnecessary parts of the protein, allowing pre-Lamin A to be henceforth known as Lamin A.
In a person without progeria, the maturation process goes on without any hiccups, and the functional protein can now go do its job (composing the structure of the nucleus). In a person with progeria, this maturation is interrupted. One of the later maturation steps cannot occur because the pre-Lamin A is missing the usual site that tells the enzyme where to cut the extra stuff out, thus the enzyme can’t do its job. This means the pre-Lamin A doesn’t get fully converted to Lamin A, causing an accumulation of the abnormal pre-Lamin A, called progerin, in the cells. Progerin gets incorporated into the nucleus’ scaffolding making cells unstable and slowly damaging them, which contributes to premature aging.
Think of it like building a house with defective support beams. The builders don’t know the beams are defective, so they use them anyway. Over time, the walls begin to warp and crack because the beams can’t do their job properly. The same thing happens at the cellular level when progerin is incorporated into the scaffold.
Worth the watch
So, there you have it! Brad Pitt and some hidden science, what more could you ask for from a movie?
I would never have thought that a movie recommendation from a friend would lead me down such an interesting path. Despite its scientific inaccuracies, “The Curious Case of Benjamin Button” is definitely worth the watch. Beyond all the science, the movie reminded me that it is never too late or too early to be whoever you want to be. And after reading this, I hope you learned that the next time a friend recommends a movie to you, give it a chance. They might not all be winners but there’s always a chance you’ll learn something.
Angelina Lapalme is a U3 BSc student majoring in Bio-Organic Chemistry at McGill University.
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