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THE SCIENCE GUY: BACK IN THE LAB FOR FA

Greetings! Class is back in session. Bill Nye is heading into the lab to explore the science behind Friedreich ataxia (FA), a rare neuromuscular disease that affects more than 5000 people in the United States. Watch the new series now and find more information about FA below or by signing up .

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00:01

MAN: I just I don’t understand.

00:03

WOMAN: Of course you don’t, Justin. And that’s why I’m breaking up with you!

00:07

MAN: I don’t get it.

00:09

WOMAN: You’re not listening to me!

00:17

MAN: Sorry, can you repeat that?

00:26

BILL NYE: What we have here is a failure to communicate.

00:32

Pull up to the lab,
It’s been a minute
‘The Science Guy’ is back
With a special mission

00:38

Friedreich ataxia?
It’s a rare disease
If you think that’s you
Let’s get up to speed

00:42

Back in the lab, back in the lab,
Back in the lab with Bill Nye
Hi Bill, hi Bill, hi!

00:52

THE VOICE: Bye Bill.

00:54

BILL NYE: Communication. Nothing works without it.

00:57

THE VOICE: I heard that in therapy.

00:59

BILL NYE: Our nervous system is a network of pathways (car SFX) that communicates between the brain and muscles. But with Friedreich ataxia, there’s a communication breakdown (mechanic SFX). Please, consider the following.

01:15

BILL NYE: Today, we’re looking at the impact of oxidative stress on the nervous system. Say, Christian, why is the system so nervous?

01:25

CHRISTIAN: Well, actually a reduction in frataxin leads to a build-up of oxidative stress, that causes a lot of cell damage, specifically in areas like the spinal cord and the peripheral nerves.

01:40

BILL NYE: Hmm. So, first, let’s look at the nervous system that’s working properly. This steel wool is the neural pathway that connects the brain to the spine.

01:51

BILL NYE: Now, let’s see what happens when we add oxidative stress.

02:02

BILL NYE: Well, I mean, it takes time. If this were a cooking show, I’d say, here’s one I prepared earlier.

02:11

BILL NYE: You see, too much oxidative stress damages nerve cells, impacting communication between the brain and the rest of the body.

02:23

PUPPET BRAIN: Hello, Spinal Cord!

02:25

PUPPET SPINAL CORD: Hey, Brain!

02:26

PUPPET BRAIN: So, what are you up to today?

02:27

PUPPET BILL: As Friedreich ataxia progresses, communication continues to break down in different parts of the body–like this.

02:41

PUPPET BRAIN: Hey, what’s the big idea!?

02:44

PUPPET SPINAL CORD: Big ears? What the what? I don’t have ears!

02:45

PUPPET BILL: When your nerve cells are damaged and aren’t communicating well, you’ll notice your symptoms getting worse–more clumsy movements, slurred speech, and so on. It’s important to note that everyone is different. It may happen faster or slower for some people.

03:00

PUPPET SPINAL CORD: I bet you think you’re soooo smart.

03:05

PUPPET BRAIN: You’ve got a lot of nerve, buddy!

03:07

PUPPET SPINAL CORD: You bet I do.

03:08

PUPPET BRAIN: I’m the brain. I’m in control. Without me you’d be NOTHING!

03:21

BILL NYE: Remember, the cells in our body have to talk to each other to function properly.

03:25

BILL NYE: But as Friedreich ataxia progresses, damage to cells causes a communication breakdown. That’s why, no matter how slow or fast you think your symptoms are progressing, you should talk to your doctor.

03:46

BILL NYE: Hey! Why can’t I connect?

03:49

THE VOICE: Sorry, I was calling my mom.

03:51

BILL NYE: Oh, that figures. Kids today.

03:59

PUPPET BRAIN: You can’t control me! Wait a minute that’s not the line in the slightest. It’s like I never even read this script. I’m a failure!

04:09

BILL NYE: There’s a communication breakdown. Please…oh boy went a little too far.

04:15

PUPPET BRAIN: Nothing I tell you, I tell you again nothing! Not one single thing!

04:22

PUPPET SPINAL CORD: Help meeeeee!

04:25

BILL NYE: Pre-circle! Purple sharpie!

04:27

PUPPET BRAIN: Don’t make me come over there and start solving algorithmic equations with the slide rule!

Episode 1 hover

Episode 3 hover

Episode 4 hover

Click to watch each episode

How does Friedreich ataxia affect the nervous system?

Oxidative stress—it’s stressful

For people living with FA, oxidative stress can lead to a communication breakdown between the brain and the nerves. Ever heard of “free radicals”? They are unstable molecules that can cause damage when left unchecked. Free radicals occur with everyday function, but can also be produced by external factors such as diet, stress, smoking, inflammation, and exposure to the sun. In people with FA, the buildup of free radicals – known as oxidative stress – is caused by a deficiency in the protein called frataxin (discussed in episode 1).

Normally, antioxidants in the body are produced by the Nrf2 pathway, which is like a big defense system. This pathway helps to neutralize free radicals. For people with FA, this defense system doesn’t function properly. Instead, oxidative stress can continue to increase and damage cells. This is what leads to the communication breakdown between the brain and the nerves.

The reason symptoms worsen over time

For people with FA, too much oxidative stress contributes to cell death, specifically within the body’s nervous system. The longer this process goes on, the more symptoms will progress. The cell damage caused by FA is irreversible, meaning loss of function is likely permanent. That’s why if you’re experiencing symptoms, it’s important to talk to your doctor as soon as possible.

Learn more about disease progression below.

Nrf2=nuclear factor erythroid 2-related factor 2.

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Brought to you by NAF and Biogen

The Science Guy: Back in the Lab for FA is brought to you by a joint effort from the National Ataxia Foundation (NAF) and Biogen, two leaders in the Friedreich ataxia space.

Bill and Christian

Meet two science guys with close ties to ataxia. Bill Nye has a long-standing relationship with NAF and has family members with a type of ataxia called Spinocerebellar ataxia type-27B (SCA27B). Bill is dedicated to helping others by spreading knowledge about ataxia. Christian M is a PhD student who has Friedreich ataxia. He studies molecular biology in hopes of one day finding a cure for his disease.

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