The Science Pawdcast

Episode 27 Season 7: Huntington's Disease Hope and Smart Dogs

• Jason and Kris Zackowski • Season 7 • Episode 27

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After a two-week hiatus dealing with shipping challenges and postal strikes, Jason and Chris return with exciting scientific breakthroughs and heartwarming pet insights. Their absence was filled with stuffy reshipping adventures and a memorable Comic-Con appearance with their super-dog companions.

The episode features a remarkable development in Huntington's disease treatment – a devastating neurodegenerative condition affecting 7 in 100,000 people worldwide. A pioneering gene therapy trial using microRNA delivered via viral vectors directly to the brain has shown unprecedented success in slowing disease progression. Patients receiving the highest dose demonstrated 75% less decline in cognitive and motor symptoms compared to untreated individuals. While invasive and still pending regulatory approval, this breakthrough offers genuine hope for patients and families who've endured decades of despair. Beyond Huntington's, this approach could potentially revolutionize treatments for other neurodegenerative diseases like Parkinson's.

The pet science segment reveals fascinating research about canine cognition, particularly how "gifted" dogs mentally categorize toys by function rather than appearance. Border collies demonstrated an ability to classify toys based solely on how they're used in play – whether for throwing or tugging – without relying on verbal cues. This suggests dogs possess cognitive flexibility comparable to human toddlers, understanding not just words but concepts. The study highlights how our canine companions don't merely memorize but actually comprehend and apply knowledge functionally, raising profound questions about their cognitive abilities.

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SPEAKER_00:

Hello, science enthusiasts. I'm Jason Zakowski.

SPEAKER_01:

And I'm Chris Zakkowski.

SPEAKER_00:

We're the pet parents of Bunsen, Beaker, Bernoulli, and Ginger.

SPEAKER_01:

The science animals on social media. If you love science and you love pets, you've come to the right spot. So put on your safety glasses and hold on to your tail.

SPEAKER_00:

This is the Science Podcast. Hi everybody, and welcome back to the Science Podcast. We hope you're happy and healthy out there. This is episode 27 of season seven. Long time no talk, Chris. We've been away from this for two weeks.

SPEAKER_01:

It's been a while. We've been super busy doing other things like reshipments, and we've been working around the clock.

SPEAKER_00:

Yeah, so we've spoken about this on social media, but to make a long story short, the Bernoulli stuffies that we had this awesome pre-sale for, and they arrived in August, and we shipped them all away on my birthday or the day after, and a whole bunch of them got rejected and customs in the states. We don't know why. We followed all the rules and like 140 of them came back. So we had to catalog and spreadsheet and then resend them and do the tariffs and the new shipping to the states. We wanted everybody to get their stuffy. And yes, Chris is right. We've been basically working every evening for a couple weeks, and the podcast had to go on the back burner. And the irony is, Chris, I was heading to the post office yesterday to drop off the remaining stuffies. And guess what happened? All across Canada.

SPEAKER_01:

You called me about this, and you said you're not going to believe it. And I said, What? And you said Canada Post has gone on strike.

SPEAKER_00:

We were very frustrated when it happened, but you and I are very stubborn, hardworking people, and we figured out a solution to it. And everybody was very actually very gracious about waiting. So some folks are gonna have to wait a little bit longer because our entire national post system is now on strike. So I can't even deliver anything, anyways. That's out of our hands.

SPEAKER_01:

Out of our hands, out of our controls. You always say there's nothing for it.

SPEAKER_00:

There's nothing for it. There's nothing we've had a really good couple of weeks with the dogs, and uh and we haven't really had pet chat to talk about it. And this isn't really the place to talk about all the fun stuff we've done with them. But just real quick, we were at Comic-Con on Saturday, and that was an amazing experience.

SPEAKER_01:

It sure was. The dogs did so great. They went as super dogs, Superman and Supergirl, and it was a lot of fun. We met some celebrities and made some memories and made our fans very happy.

SPEAKER_00:

Yeah, it was an awesome time. Okay, what's on the show this week? In Science News, there was a really big story about Huntington's disease, and I thought we would sink our teeth into that. And in Pet Science, a really cute study about I guess they're gifted dogs and how they process language. I don't know, I don't know if our dogs would be considered gifted. We're talking about those border collies that know hundreds of words. I don't know if our dogs would be considered gifted. They're smart. I don't know, I don't know if they'd be in immense quality, though.

SPEAKER_01:

No. But although I was very impressed that you can say find your leash, and Bernoulli does it.

SPEAKER_00:

Yeah. He's way smarter than he looks. He just looks like a happy lug. All right, on with the show because there's no time like Science Time. This week in Science News, let's talk about Huntington's disease. Now, you've probably heard of that before, and it's been a hot minute since I've had to explain to students what Huntington's disease is, but it's a mutation in the Huntington gene, and the mutation leads to an abnormally long protein that becomes toxic. It's autosomal dominant. So that means a child of somebody with Huntington's disease has a 50% chance of inheriting. So it is extremely genetic. Now, some of the symptoms involve involuntary movements. Your muscles get stiff and you have difficulty with voluntary movements. You have trouble speaking, swallowing, and have coordination issues. And it's progressive. You have cognitive decline with psychiatric symptoms. So the disease is fatal, which is really sad, and currently has no cure. And the kicker to it is this is the thing that I have to explain to students because they're always like, if you're if you have Huntington's disease, why don't you just choose not to have kids? Because it's a flip of a coin to pass it on. And the problem is patients develop the symptoms in mid-adulthood. You probably have started a family by the time you're diagnosed with it. And then it's a very progressive disability and an early death. About seven in 100,000 people suffer from Huntingdon's disease.

SPEAKER_01:

But Jason, there's been an experimental gene therapy trial, and it's pretty much a breakthrough announcement. On September 24th, so very recently, some results from a small clinical trial of gene therapy were announced. And you know what? It generated some excitement and some hope. Since, as you said, Huntington's disease usually carries only grim news. Yeah, it's bad. It's bad news. So how the therapy works is the therapy uses microRNA, which are tiny RNA fragments that actually prevent the Huntington protein from forming. And these RNA molecules were delivered using a viral vector, which was injected directly into the brain, which is different than the previous methods that they use to maybe combat or study Huntington's disease. And so what happens is the virus, in quotations, infects the neurons and reprograms them to continuously produce microRNA that blocks that tuck that toxic Huntington production. So preventing that long protein from forming. And as I said, this approach is different from earlier trials because the RNA was injected into the cerebrospinal fluid, which actually failed to reach critical areas of the brain.

unknown:

Right.

SPEAKER_00:

And you had mentioned that it's a viral vector. So just quickly, if that term is confusing to you, that's something that scientists have borrowed from viruses, and it delivers genetic material into cells. Remember, viruses are super good at sneaking their genetic instructions into other cells. And if we can modify them to carry instructions instead of good instructions instead of harmful ones, those instructions can get it's almost like a heist. Like they can ocean eleven themselves through the cell membrane and into the main part of the cell. And that's what you mentioned. They're continuously producing those RNA blockers. The trial details are interesting. It's a very small study of 17 people with early Huntington symptoms. The surgery was no joke. It lasted 12 to 18 hours. And the viral vectoral payload was injected into three different brain sites per hemisphere. Of those 17, 12 patients were monitored for 36 months, and they gave them different tests to determine their progression of disease, like motor control, their attention, their memory, and how well they did at daily life skills. And Chris, the results were very cool.

SPEAKER_01:

They were very cool. So we would like to say it was a small sample study, but it does show promise because it isn't a cure, but the disease progression was slowed. And patients on the highest dose showed about a 75% less decline in cognitive and motor symptoms compared to the untreated patients. And some patient symptoms actually appeared to stabilize. So sharing the stories, one patient who was previously unable to work actually returned to his IT job a year after going through therapy. And others that were expected to be wheelchair bound were still walking after three years, which is incredible.

SPEAKER_00:

Yeah, so this is a really good news study. Yeah, this is such a good news study. Huntington's disease is just devastating.

SPEAKER_01:

Yeah, Jason, for sure. There were some limitations and definite challenges to the method that they used. Of course, the limitation is the very small trial, trial size of 17 patients, and their results actually are not yet peer reviewed or published. And you mentioned the brain surgery being no joke. It was invasive. It's invasive brain surgery. And unfortunately, treatment will likely be extremely expensive because each dose was custom made and the regulatory approval is still pending. It hasn't been given a rubber stamp. But there's some broader implications that are positive because this could inspire similar approaches for other neurodegenerative diseases like Parkinson's and other viral vector therapies that are already approved for rare disorders. MicroRNA delivery is considered relatively easy to adapt to these other conditions.

SPEAKER_00:

So the this could be the low-hanging fruit to nail off some of those other degenerative diseases, slowing their, slowing the decline of people that have them.

SPEAKER_01:

Yeah, and it gives hope. Patients and their families did express hope after having decades of despair. And that's what's still in Pandora's box.

SPEAKER_00:

We've had a hard week, and the news out there sometimes isn't good. This is definitely a good news story. That's science news for this week. This weekend, pet science. Let's talk about some really smart dogs and how they process language. How many words do you think Bernoulli, Bunsen, and Beaker know? What do they know a lot of words? I think they do.

SPEAKER_01:

I think they do. I think they know the keywords like walk and water and food and bathroom and outside and want to go on the deck, and I'm gonna spray you with water, and Bunsen will run away. Because I was just thinking today they're really muddy. When you tell them in advance, some of them they're like, I don't want to really really want to do that.

SPEAKER_00:

The creek is really muddy. I don't know which of the three of them knows the most words. I want to say Bernoulli, honestly. Bernoulli may know the most words of the three.

SPEAKER_01:

He might. I don't know. They all we just talk to them and we play with them. And you know what? I think that might be because Bernoulli is the most interactive with toys. Whereas Beaker and Bunsen will just leave them. Like Bunsen's like, I have zero interest in that until I do, but that's that interest is few and far between. And Beaker likes a few select toys, but Bernoulli actually engages in play. And he's really quite funny. When he was getting picked up from Doggy Daycare, they had a basket of free toys. And once he was told once that he could have some of these free toys, he took one every day. And I hated that. He had one every day and he just loved it. So now you go pick him up and they have a table full of baby clothes because one of the trainers there, she's having a baby. So they have this baby draw for guess the weight, all that stuff, and these wonderful clothes that are on the table. You know what? Bernoulli doesn't differentiate between those toys that were free for the taking and the little baby onesie that he definitely took the other day. And I was like, oh no. It was funny, but also not.

SPEAKER_00:

That's a Bernoulli thing.

SPEAKER_01:

Such a Bernoulli thing. And then I got it away from him, and then he went back to the table and got a different one. So it was a thing.

SPEAKER_00:

He he is he's crafty. He is he's into things, yeah. All right, let's get to the study. This study shows that some dogs actually mentally label toys based on how they're used in play, so they sort them by function, not just by appearance or verbal cues. And this study comes from current biology, really recently, September 18th. Now, this was sparked by an observation. A dog in another study categorize toys into groups like ball, rope, and ring. And remarkably, the dog could also classify toys she's net she'd never seen before into those groups. So the researchers asked then can dogs also categorize toys strictly by function rather than what they look like.

SPEAKER_01:

Of course, you mentioned the study participants were the gifted word learner dogs, mostly border collies. They're so smart, and they have an unusually large vocabulary of toys that they use that they've learned naturally at home. Because I think the border collies play with their toys and they really enjoy that. They're like fun and they're like, please play with me, otherwise, I'm going to eat your drywall.

SPEAKER_00:

Yeah, they need a job and they need mental stimulation.

SPEAKER_01:

Um, exactly.

SPEAKER_00:

Bunsen and Bernoulli just need the couch, like they just need to go to sleep, they just power down.

SPEAKER_01:

They do, but I think we would be very busy with a border collie. Um, we would be over the top. It would be what? Oh my goodness. Eleven dogs were recruited for this study, and then seven completed the project. There was a few there that didn't make the cut. And I know, and it was fun. The experiment was conducted in dogs' home environments, and that ensure and that ensured natural play. And so the first thing that they did was functional training. So the owners played either throw or fetch or pull, like a tug-of-board game, with their dogs, um, using plushies, squeakers, ropes, a variety of toys. And then each toy was randomly assigned to one category, so like the throw category or the pull category, regardless of its physical traits. And then owners named the activity out loud, right? Giving a name to it, like throw or pull, while they played. And then in the next step, they tested with novel toys, so the toys that dogs had not seen before, by introducing those new toys and playing either fetch or tug with those toys, but they did not name the activity allowed. And so the dogs had to rely only on the experience of the function, not the verbal or the visual cues.

SPEAKER_00:

And then this new toy, like the new toy, they just played tug with it, and the dog just had to know, oh, this is a tug toy, and they never were told.

SPEAKER_01:

And then step three, or the last part of the study, was owners were they said, but and then lastly, step three was a retrieval task where owners asked their dogs, hey, bring me a throw or bring me a pull from a pile of old and new toys. And the test would be would dogs apply the functional category to the toys they had never heard labeled before. So would they realize, oh, that was a new toy, and I played tug with it?

SPEAKER_00:

It's a very human thing, right? If you get a bunch of kindergartner kids together and you're like, these are scissors and they cut, and then you give them something that looks like scissors, I'm sure they'd be able to classify them, right? That's a human thing that humans are really good at doing is classifying a bunch of stuff together as function. So here come the results, Chris. The dogs did great. Out of 48 trials, dogs selected the correct novel toy an average of 31 times. And the mistakes mostly happened when dogs grabbed an old toy they had already heard labeled. Now that's not a hundred percent. 31 out of 40 48 is about a 65% rate that's much higher than a guess. And the findings were enough to show that dogs generalize function categories from familiar toys to new ones without verbal instruction. Very cool.

SPEAKER_01:

So it's a significant study though, Jay, because it's pretty novel. Previous studies relied heavily on trained captive animals, whereas this one was testing categorization by function in the natural home setting with a pet dog versus a heavily trained captive animal. And it carefully, the study actually carefully ruled out reliance on physical features or other cues, and they actually assessed the dog's cognitive level. So the dog's skill was compared to that of a human toddler. Aww. I know. So that suggests dogs don't just memorize words, but they can use them in a flexible functional way.

SPEAKER_00:

That's cute. I've heard that so many we've done a lot of dog studies, and that's the capacity that your dogs have. They're a toddler, they're a baby, not a baby, but they're a toddler. Yeah, and toddlers are toddlers will surprise you with what they know, but they're not an adult. So you that's maybe that's why we love them so much. Because toddlers are a lot of fun, but they're a lot of work too.

SPEAKER_01:

Exactly.

SPEAKER_00:

I was never more tired. I keep saying this. I was never more tired than when Adam was a toddler, but he was also a lot of fun. Future studies may explore other categories beyond throw and pull. Maybe what's another category of frisbee? I don't have no idea. I guess they'll have to come up with other categories. And it raises a broader question about how dogs recognize, remember, and infer. And these are all signs of complex cognition. We know dogs are smart, and I guess we're just finding out that they're as smart as some humans. If the humans were toddlers. Alright, that's Pet Science for this week. That's it for this week's show. Thanks for coming back week after week to listen to the science podcast. And a shout out to all the top dogs. That's the top tier of our Patreon community, The Popack. You can sign up in our show notes. All right, Chris, let's hear those names that are part of the top dogs.

SPEAKER_01:

Amelia Fettig, Re Oda, Carol Haino, Jennifer Challenge, Linnea Janet, Karen Cronister, Vicky Oteiro, Christy Walker, Sarah Bram, Wendy, Diane, Mason and Luke, Helen Chin, Elizabeth Bourgeois, Marianne McNally, Katherine Jordan, Shelly Smith, Laura Steffenson, Tracy Leinbaugh, Anne Uchida, Heather Burbach, Kelly, Tracy Halbert, Ben Rather, Debbie Anderson, Sandy Brimer, Mary Rader, Bianca Hyde, Andrew Lynn, Brenda Clark, Brianne Hawes, Peggy McKeel, Holly Birch, Kathy Zirker, Susan Wagner, and Liz Button.

SPEAKER_00:

For science, empathy, and cuteness.