Good morning. Few medical diagnoses are as brutal and devastating as Huntington’s disease.
For decades, those who inherited this cruel condition faced only despair. There was no cure. Symptoms began with mood swings and depression, then progressed to a loss of movement, followed by dementia, paralysis and, ultimately, death. Some patients died within a decade of diagnosis.
Finally, a ray of light has burst through. For the first time, Huntington’s disease has been treated successfully through gene therapy, slowing the progress of the disease by 75% in patients after three years in a groundbreaking trial.
It is one of several extraordinary medical advances announced in recent months, alongside new possible treatments and diagnostic tests for Alzheimer’s, heart disease and stroke.
For today, I spoke with Hannah Devlin, the Guardian’s science correspondent, about the exciting research behind this desperately needed good news, and why cuts to scientific research could put future progress at risk. That’s after the headlines.
Five big stories
France | The former French president Nicolas Sarkozy has been found guilty of criminal conspiracy, and given a five-year prison sentence.
UK politics | All working adults will need digital ID cards under plans to be announced by Keir Starmer, in a move that will spark a battle with civil liberties campaigners.
Middle East | The White House is backing a plan that would see Tony Blair head a temporary administration of the Gaza Strip – initially without the direct involvement of the Palestinian Authority (PA), according to Israeli media reports.
US news | James Comey, the former FBI director and one of Donald Trump’s most frequent targets, was indicted on one count of making a false statement to Congress and one count of obstruction of justice, in the latest move in the president’s retribution campaign against his political adversaries.
UK news | The crown court backlog in England and Wales has hit a new record of almost 80,000 cases, while wait times for trial dates have reached up to four years.
In depth: ‘Symptoms in the prime of your life’
Huntington’s disease is a genetic disease that runs through families. It is caused by a single mutation of a gene that makes a protein called huntingtin.
Huntingtin is an essential protein in the brain, but in people who have the mutation, the brain makes a toxic version of it that kills brain cells, Hannah Devlin told me. Between 6,000 and 10,000 people in the UK have the diseases, and another 20,000 are carriers of the faulty gene, which means they are likely to develop it.
“If you’ve got the gene, you tend to get your first symptoms in the prime of your life – in your 30s and 40s. Then you’ll undergo a mental decline, which is a form of dementia, as well as motor issues; including jerky movements, losing your balance and gradually becoming paralysed,” Hannah said. “It’s a truly devastating condition, and one of the really awful things about it is if you’re a gene carrier, your children will have a 50% chance of also having the gene.”
Over the last 10 years, Hannah has reported on notable progress in the scientific community’s understanding of how the mutant gene causes the disease.
She said: “There has been growing hope that a treatment was on the horizon, but practically, there hasn’t been anything for patients. Until now.”
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A one-off injection
The successful trial used gene therapy to silence the mutant gene so the brain stops producing the toxic protein.
The challenge for researchers was overcoming the blood-brain barrier. “While drugs can flow around the body, the brain has a special barrier that stops that from happening. So getting any treatment into the brain is itself a massive hurdle,” Hannah said.
The breakthrough treatment worked by delivering a small piece of genetic material attached to a harmless virus. Scientists infused the virus directly into the brain. Once inside, the virus entered neurons and delivered the genetic material that silences the mutant gene, stopping it from producing the toxic protein.
The procedure is incredibly complex and delicate, Hannah said. “You have to infuse this virus into the brain very slowly, over 12 to 20 hours, through microcatheters put into the two brain areas that are most affected by Huntington’s.”
She added that this gene therapy unit is remarkable because it is effectively a one-off injection. “So they had the surgery, had this treatment delivered once, and then they were followed up for three years to see whether it altered the progress of the disease.”
The research shows that the gene therapy slowed the progress of the disease by 75% in patients after three years.
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Ripple of activity
While political leaders appear intent on delivering doom and gloom, the scientific community seems determined to do the opposite.
The breakthrough treatment for Huntington’s disease is the latest in a string of remarkable advances, including promising new therapies and diagnostic testing for Alzheimer’s, heart disease and stroke.
“We have seen some really exciting developments in Alzheimer’s, including the approval of a couple of exciting drugs that could slow progression of the disease. They’re not currently funded by the NHS, but it’s been seen as a big development,” Hannah said.
She also pointed to notable breakthroughs in how Alzheimer’s is diagnosed, including a £100 blood test and a three-minute brain scan, which medics hope could transform diagnosis of the devastating condition. These diagnostics could be critical to getting a much faster diagnosis, which is crucial to treating the disease.
“Often when one breakthrough happens, it prompts this ripple of activity and things can suddenly move quite fast in an area where it feels like there was very little progress for years,” Hannah told me.
The other big developments recently have been in treatments for heart disease and stroke. Researchers found that a blood-thinning drug could be more effective at preventing heart attacks and stroke than aspirin, which has been the standard for decades.
“A lot of this is off the back of big investments into health research,” Hannah said, but that now appears under threat in some corners of the globe.
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A bright future, dimmed
The breakthrough in treating Huntington’s disease would not have happened without public funding.
“This is work that has been going on for more than a decade at UCL [University College London]. This is an absolutely world-leading team that has benefited from public funding into how to study this awful disease. It’s now being fast-tracked in the United States because of this incredible reputation that the FDA [Food and Drug Administration] has with speed and their links with the research community,” Hannah said.
But that is now under threat. There have already been major staff layoffs at the FDA, as well as a proposed cut of more than 40% to the National Institutes of Health budget amid what has been called an “assault on science” by the Trump administration. And as I explored in my previous first edition on the impact of US cuts on vaccine research, these decisions will be felt across the world.
“If you fast forward 10 years, some of the research that’s been cut will translate into fewer treatments that could help with some of these really awful diseases. In some cases, researchers are just on the cusp of being able to treat some really devastating diseases so it does feel depressing that these cuts are happening in parallel,” Hannah said.
Time will tell whether other countries or global institutions step in to fill the gap left by the US. Until then, I’ll be here to deliver these happy First Editions, only tempered by a bit of caution at the end.
What else we’ve been reading
I was depressed by how quickly Jeremy Corbyn and Zarah Sultana’s Your Party seemingly fell apart. But this column by Andy Beckett has convinced me to hold my horses before being sure it’s all over for them. Poppy
A fleet of civilian vessels is sailing to Gaza. It is believed to be the largest convoy in history to traverse the Mediterranean Sea, with a mission to feed starving Palestinians. David Adler, who is on the boat, explains why he is taking part. Aamna
I’m not an opera person, but I loved the images in this photo essay by one of the Guardian greats David Levene and a cast of other amazing photographers, an inside look at the English National Opera’s season-opener. Poppy
I am so buzzed to see the England Women’s Rugby team in the World Cup final. They are sticking with the same match-day squad that defeated France to take on Canada, with captain Zoe Aldcroft insisting “now is our time”. Let’s go! Aamna
The two-child-benefit cap has become a bit of a political football in recent years, so I felt this video report, showing its real-life impact – devastating families and forcing them into humiliating compromises – was much needed. Poppy
Sport
Fotball | John McGinn’s first-half strike earned Aston Villa their first win of the season with a 1-0 victory over Bologna in their Europa League opener at Villa Park. Former Celtic striker Oh Hyeon-gyu scored after missing an earlier penalty as Genk beat a 10-man Rangers 1-0 in their Europa League opener at Ibrox.
Football | William Saliba, the 24-year-old centre-back who has been a mainstay of Mikel Arteta’s Arsenal since the 2022-23 season, has agreed a new five-year contract with the team, despite Real Madrid interest
Golf | Club-tangling rough, narrow fairways and a tough workout lie in wait for the USA and Europe in the 45th Ryder Cup, as shown in our hole-by-hole course guide.
The front pages
The Guardian leads with “Microsoft blocks Israel’s use of its technology for mass surveillance”. The Telegraph has “Starmer: Labour got it wrong on migration”. The i says “‘BritCard’ digital ID will be made law for all adults in bid to tackle small boats”. The Times reports “Digital ID card scheme will confirm right to work”. The Mirror goes with “Playing the BritCard”, while the Mail has “Show us your (digital) papers”. Finally the Financial Times leads with “Burnham borrowing spree would rock gilts and hurt sterling, investors warn”.
Something for the weekend
Our critics’ roundup of the best things to watch, read, play and listen to right now
TV
House of Guinness | ★★★★★
The family here is not a crime family: we are in Dublin in 1868, where Guinness is so ubiquitous that the unimaginably wealthy Guinness family run the city. But managing the factory that dominates the landscape is the fearsome Sean Rafferty (James Norton), an arch schemer whose currency is violence. He introduces himself by issuing a rallying call to the company workers, exhorting them to crush an anti-Guinness street protest then leading the way himself, gleefully swinging a hunk of hard factory iron. Jack Seale
Music
Olivia Dean: The Art of Loving | ★★★★☆
Dean’s 2023 debut album, Messy, attracted respectful but mixed reviews and did respectable, rather than remarkable, business. While you wouldn’t describe The Art of Loving as a complete reinvention, it certainly constitutes a noticeable rethink. It expunges most of the cliches of Dean’s debut – or rather quarantines them on a track called Close Up – and instead looks for inspiration to music that emanated from recording studios in 70s LA. Alexis Petridis
Film
Sane Inside Insanity: The Phenomenon of Rocky Horror | ★★★☆☆
Andreas Zerr’s unofficial documentary charts the 50-year cult of Richard O’Brien’s beloved classic, from its chaotic early days upstairs at the Royal Court theatre. With archival footage, and cast, fans and producers among the talking heads, it shows how a “shitty fucking movie” became an accidental phenomenon that imitators could never match. Catherine Bray
Theatre
Measure for Measure (Royal Shakespeare theatre, Stratford-upon-Avon) | ★★★★★
Emily Burns’s razor-sharp revival cuts away the comic subplots to deliver a tense, streamlined account of Shakespeare’s play about sexual hypocrisy and power. With slick modern dress, a glass-and-chrome set evoking a political battlefield, and a montage of real-world scandals, the parallels with today are stark. Tom Mothersdale’s Angelo is both appalling and human, while Isis Hainsworth’s Isabella grows from wide-eyed novice to a mighty force. Arifa Akbar
Today in Focus
‘Like Amazon Prime but with human beings’: inside Trump’s deportation machine
Data leak gives Guardian US investigations team an unprecedented look into Trump’s deportation regime – and how people are seemingly being ‘disappeared’. Oliver Laughland and Maanvi Singh report.
Cartoon of the day | Ben Jennings
The Upside
A bit of good news to remind you that the world’s not all bad
A much-loved ash tree on Argyle Street in Glasgow has won the tree of the year competition. The towering Victorian-era tree beat the “King of Limbs” oak in Wiltshire and other majestic specimens in a contest organised by the Woodland Trust. The ash stands 75ft (23 metres) tall on one of the Glasgow ’s busiest roads and has survived war, redevelopment and even ash dieback disease. Locals take pride in its elegance – it has been featured in a book and is celebrated in a nearby pub. This urban survivor will represent the UK in the European contest next spring.
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Bored at work?
And finally, the Guardian’s puzzles are here to keep you entertained throughout the day. Until Monday.
