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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

BILL NYE: Curiosity! It’s the reason we have things like space travel (rocket SFX), electricity (lightbulb SFX), and… a deeper understanding of genes!

00:12

BILL NYE: Not those genes! I mean the genes in our DNA.

00:13

THE VOICE: Whoops.

00:17

BILL NYE: Over the last several decades, we’ve learned a lot about the science behind our genes.

00:24

BILL NYE: A rare neuromuscular disease called Friedreich ataxia gets passed down through the genes. Our curiosity has taught us a lot about it, but it’s going to be your curiosity that drives us all forward.

00:37

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

00:42

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

00:47

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

00:55

THE VOICE: Ooh, this’ll be good!

00:57

BILL NYE: So, Friedreich ataxia, where has our curiosity taken us so far? Let’s rewind.

01:12

BILL NYE: Ha ha. It’s pie!

01:17

2D BILL NYE (V.O.): Friedreich ataxia is caused by a mutation in the gene that creates frataxin. A DNA sequence called the GAA triplet repeats way more than it should, impacting frataxin production. Frataxin helps create energy, and without it, it can lead to all kinds of trouble. You may notice symptoms like frequent falls, clumsiness, tiredness, and slurred speech.

01:47

2D BILL NYE (V.O.): Let me ask you this: Why don’t GAA triplets make good storytellers? Because they keep repeating themselves. Ha ha. Repeating themselves? See what I did there?

02:02

2D BILL CLONE: Bye Bill.

02:05

THE VOICE: Mmm. That wasn’t great.

02:10

PUPPET BRAIN: Hey! What’s the big idea!

02:12

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

02:15

PUPPET BILL (V.O.): As Friedreich ataxia progresses, communication continues to break down in different parts of the body. When your nerve cells are damaged and aren’t communicating well, you’ll notice your symptoms getting worse.

02:29

PUPPET BRAIN: WILL YOU JUST GET BACK TO WORK ALREADY?

02:33

PUPPET SPINAL CORD: WHY DON’T YOU DO IT YOURSELF FOR ONCE? ALWAYS BOSSING ME AROUND.

02:37

PUPPET BRAIN: UGH YOU GIVE ME SUCH A PAIN IN THE AMIGDELA.

03:42

ROMAN WOMAN: Is it time for my bath? I think we’re way over.

02:46

BILL NYE (V.O.): For someone with Friedreich ataxia, a reduction in frataxin production may cause abnormal mitochondrial function in the heart cells.

02:54

CLAY BILL (V.O.): People might not even notice it’s happening, but the walls of the heart can get thicker and thicker over time. This can make it difficult for the heart to pump blood effectively.

03:05

2D BILL NYE CLONE: Hi Billlllll. Oh Hi Bill!

03:16

CHRISTIAN: Oh hi Bill!

03:18

PUPPET BILL (V.O.): Well if it isn’t my favorite friendly expert! So, Christian, what’s it like being a researcher who studies Friedreich ataxia, while having Friedreich ataxia? Did your diagnosis inform your interest in science at all?

02:32

CHRISTIAN: Well, you know, I’ve actually always been very interested in science, specifically in entomology. I’ve always really enjoyed the intricacies of small systems. But when I learned about molecular biology, I knew that there was a whole world of things that were much smaller. And, I would say that studying FA has been very helpful in understanding the disease and helping me in my journey with FA, and staying in the know.

04:02

PUPPET BILL: Thanks Christian. Before we go, do you have anything you’d like to tell people who may be wondering if they have Friedreich ataxia?

04:10

CHRISTIAN: I think the first thing I would tell them, Bill, is to go see a doctor, and ask them a little bit more questions. And then, if they are diagnosed, remember that you’re not alone, that there’s the whole community of people out there willing to help you. And I think, the biggest thing I would tell them is just, remember that you’re not alone.

04:31

THE VOICE: We’re back, Bill.

04:32

BILL NYE: Remember, at this moment in time, Friedreich ataxia is irreversible, but the science is evolving, and there are treatments available that could help.

04:42

BILL NYE: So, if you think you might have Friedreich ataxia, talk to your doctor. Staying curious is how we move forward. Now let’s test those genes!

04:47

BILL NYE: … I just really love genes.

05:02

PUPPET BRAIN: Will you just keep… How about if I do it right?

05:05

BILL NYE: So, if you think you might have Friedreich ataxia, let’s look at this camera.

05:10

PUPPET SPINAL CORD: Keep your hemispheres to yourself!

05:12

PUPPET BRAIN: I would love to!

05:13

BILL NYE: I just really love genes… oh shoot!

05:17

PUPPET SPINAL CORD: AHHHHHHH!

05:19

PUPPET BRAIN: I’m in charge! I’m losing my can too!

05:25

BILL NYE: Ahhh that sucked.

05:28

PUPPET BILL: Alrighty, bye.

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Let's recap

In our last episode, Bill and Christian recap what we’ve learned and show us how curiosity plays a role in managing Friedreich ataxia. While curiosity has led us to learn a lot about FA, it is your curiosity that will push us forward. So, if you think you might have FA, don’t wait to talk to your doctor. The most important things to remember when thinking about FA are:

Most people start experiencing symptoms around or before puberty, but the age of onset can vary. Some people with FA start experiencing symptoms in their 30s or even later
FA is progressive: symptoms will worsen over time, and loss of function is likely permanent
The right genetic test can quickly identify FA. The only way to confirm an FA diagnosis is with a test that analyzes GAA triplet repeats in DNA
There are ways to manage FA. If you suspect you may have FA, talk to your doctor

Learn more about managing FA below.

Now, let’s meet some folks who champion the power of staying curious.

About Bill Nye

Bill Nye is a scientist, engineer, comedian, author, and inventor. His mission is to help foster a scientifically literate society so that people everywhere understand and appreciate the science that makes our world work. Making science entertaining and accessible is something Bill has been doing most of his life, most notably with the 90s TV program Bill Nye the Science Guy.

Bill has close ties to the ataxia community; he lost his father to spinal cerebellar ataxia (SCA27B) and has many family members who are affected, including his sister and brother. This has led him down the path of curiosity to raise awareness for the condition. Now he turns his focus to Friedreich ataxia.

About Christian M

Christian M is a PhD candidate at the University of Florida in Genetics and Genomics. His research explores how gene expression differs in people with FA. Interested in biology from a young age, Christian started to explore the intricate world of molecular biology when he was diagnosed with FA. Now, he’s conducting research on FA in hopes of one day finding a cure for his disease.

About NAF and Biogen

National Ataxia Foundation (NAF)

National Ataxia Foundation (NAF)

The National Ataxia Foundation (NAF) was established in 1957 to help persons with Ataxia and their families. Our mission is to accelerate the development of treatments and a cure while working to improve the lives of those living with Ataxia. NAF’s vision of a world without Ataxia will be accomplished through our primary programs of funding Ataxia research, providing vital programs and services for Ataxia families, and partnering with pharmaceutical companies in the search for treatments and a cure. We work closely with the world’s leading Ataxia researchers and clinicians, promoting exchanges of ideas and innovation in Ataxia discovery.

Biogen

Biogen

Founded in 1978, Biogen is a leading biotechnology company that pioneers innovative science to deliver new medicines to transform patients’ lives. Biogen has spent more than a decade advancing research and treatments for rare neuromuscular diseases, including spinal muscular atrophy (SMA), amyotrophic lateral sclerosis (ALS), and Friedreich ataxia (FA). Biogen is proud to partner with NAF and Bill Nye to help raise awareness and spread the word about FA.

Learn more about Biogen's FA efforts below.

Receive emails about FA

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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