‘Major Biomedical Triumph’ as Scientists Restore Seven-Year-Old Boy’s Skin

Image: CMR Unimore

Junctional epidermolysis bullosa is the sort of rare disease you are probably lucky to have never heard of. An often lethal genetic condition, from infancy it plagues its victim with painful blisters all over the body that causes the skin to become extremely fragile.

In a major medical breakthrough, on Wednesday Italian researchers announced that they were able to almost entirely reconstruct the skin of a seven-year-old boy afflicted with JED—and they used gene therapy to do it.


It is a breakthrough that not only signals a potential curative treatment for a painful, heartbreaking disease, but demonstrates the great power that new technologies like gene therapy and stem cells may hold to address genetic conditions previously written off as hopeless.

Typically, the way JEB is treated is to manage wounds and infections as they crop up, taking care to treat the body’s extremely fragile skin delicately. In the new research published Wednesday in the journal Nature, researchers from Italy’s University of Modena and Reggio Emilia describe using gene therapy to regenerate a fully functional epidermis covering approximately 80 percent of the body in a young boy with a life-threatening case of the disease.

Illustration showing how gene therapy was used to treat a skin disease. (Image: Nature News & Views)

JEB is caused by mutations in the genes LAMA3, LAMB3 and LAMC2 which affect the production of a protein called laminin-332. This protein is essential to developing a healthy epidermis, and without it the skin is fragile and prone to infection and skin cancer.


The young patient at the center of the new study had a mutation to the gene LAMB3. To fix this, researchers took skin cells from a part of the patient’s body that was not blistering. They then cultured those cells and genetically modified them to contain a corrected copy of the LAMB3 gene. They then grafted these new skin cells onto the patient’s body. Over the course 21 months, four-fifths of the boy’s epidermis regenerated, creating a new layer of healthy skin. Testing showed that the grafted skin cells were regenerating within the patient’s body effectively.

“This report is a major biomedical triumph, combining gene therapy and stem cells,” said Eric Topol, a geneticist at Scripps Research Institute who was not affiliated with the study. “While only a case report, the seven-year-old boy was in a life-threatening condition and this intervention can be credited to saving his life.”

In previous studies, researchers had successfully reconstructed small areas of patients’ skin, but with this new approach, the researchers were the first to almost entirely reconstruct a patient’s skin.


“Skin diseases are well suited for gene therapy since the tissue is directly accessible,” Topol told Gizmodo.

Topol said the only downside was that in the long term, it’s possible that the condition could reoccur or cause troubling side effects like cancer. More extensive studies will be needed to see how the treatment works over the long haul and in other patients.

Recently, gene therapy has made many impressive strides. Just last month, an FDA panel approved gene therapy to cure a rare eye disease.


from Gizmodo http://bit.ly/2zrsTi1

Deep knowledge and free sounds for the PO-32 pocket drum machine


Teenage Engineering’s PO-32 is a powerful drum synth, literally in a calculator form factor. Now you can learn more – and update its sounds – from YouTube.

I do mean literally install sounds from YouTube. You see, the PO-32 uses a bizarre and clever update mechanism where it can absorb signals from sounds – meaning you can play the video and load sounds onto the hardware, a bit like those evil alien hypnosis tricks in old scifi movies.

Teenage Engineering and their PO-32 we already knew were great – and the hardware is well under a hundred bucks. But it takes the singular epic powers of music technologist Jakob Haq for us to unlock still more greatness.

First, the “EPIC” (his words, though I agree) review + tutorial:

Next, get some sounds. There’s The haQ attaQ TeQno TroniQ drum pack, for instance, with 16 new sounds and 4 “techno type” pattern chains:

But there’s more. As of this writing, he’s got a playlist with some seven sound banks, by genre. Good stuff:

Genius stuff. More on the product:


And you can find a bunch more links in Mr. Haq’s videos – including, not to be forgotten, that important Patreon link so you can support his work.

The post Deep knowledge and free sounds for the PO-32 pocket drum machine appeared first on CDM Create Digital Music.

from Create Digital Music http://bit.ly/2hUjWEm

This is the airline you should fly if you want a terrific start to your Hawaiian vacation (HA)


Hawaiian Airlines Review

  • I recently flew Hawaiian Airlines from Honolulu to New York.
  • The scene at Honolulu International Airport was a bit hectic.
  • But the Hawaiian Airlines flight itself was terrific.

Founded in 1929, Hawaiian is one of the oldest airlines in the world. Yet, unless you’ve flown into or out of Hawaii, it’s safe to say most of us have not had the pleasure to experience one of the airline’s delightfully pleasant flights.

As much as Emirates is a globe-trotting ambassador for Dubai and Qantas for Australia, Hawaiian serves as the flying embodiment of America’s 50th state.

These days, the airline is riding high and expanding to new destinations across Asia. Hawaiian is also welcoming a new fleet of Airbus A321neo jets that will help it grow to new markets in the US.

Even though Hawaiian suffered from financial problems during the early 2000s, the airline has been resurgent over the past dozen years under the leadership of CEO Mark Dunkerley. In 2016, Hawaiian’s annual profits surged 48% to $280 million while being named the world’s most punctual airline by aviation intelligence firm OAG.

Recently, I had the chance to experience Hawaiian Airlines on a flight home to New York after a spending a couple of days in Honolulu on business.

I took Hawaiian Flight 50, non-stop service from Honolulu International Airport (HNL) to JFK International, that was scheduled to take off at 3:15 pm and land in NYC at around 7 am. Since Business Insider was kind enough to pick up the tab for the 10-hour-long flight, I felt it was a good idea to show up to our Manhattan office at 9 am. This meant I actually had to get good sleep on the flight if I had any chance of being a functioning human being at work the next day.

But I digress. Here’s a closer look at my experience on board Hawaiian Flight Five-O:

SEE ALSO: I flew JetBlue for the first time and finally understand why it’s one of the best airlines in the world

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After two days in the tropical sunshine on the island of Oahu and a one-on-one interview with Hawaiian Airlines CEO Mark Dunkerley (keep an eye out for that story), it was time to return to the concrete jungle; New York City.

I arrive at the airport for my 3:15 pm flight shortly after noon. Since this was my first time flying out of HNL, I figured better safe than sorry. But the scene I encountered at the terminal could be charitably described as a ‘hot mess.’

I stepped off the shuttle and onto the curb in front of the terminal only to be confronted by a long snaking line of people. Although the queue seemed to be moving at a decent pace, there were no signs around to identify where the line was headed. There were also too few airport and airline staff members to effectively manage and clearly communicate with the crush of people. This is a significant problem when a large portion of those traveling through the facility are tourists with limited command of the English language.

After a minute or two, I managed to flag down a staff member who pointed me in the right direction. With that said, all of the staff I did encounter at the airport were courteous and helpful. There just weren’t enough of them around.

As it turns out, the massive line was for the TSA security checkpoint. So I walked into the terminal to drop off my checked luggage.

While I’m aware that HNL is in the midst of a $2.7 billion renovation, the chaotic scene was more than a bit concerning because there frankly shouldn’t have been one. My flight was on a random Thursday afternoon during a not-so-busy part of the travel calendar. I can’t imagine what the scene would be like during peak travel season in the summer and winter months.

I spoke with one of my contacts at Hawaiian Airlines after my flight and was assured that the airline is aware of the situation and is working hard to improve conditions at the terminal.

After making my way into the terminal, I made a beeline for the Hawaiian Airlines automated kiosks. As with most of these modern systems, the process was pretty straightforward. I put my bag on the luggage scale and the kiosk spat out a baggage tag.

See the rest of the story at Business Insider

from SAI http://read.bi/2yHlThU

Here are the ages your brain matures at everything


Neuroscientists are confirming what car rental places already figured out — the brain doesn’t fully mature until age 25.

Up until this age, the prefrontal cortex — the part of the brain that helps curb impulsive behavior — is not yet fully developed. Some scientists say this could illuminate a potential factor behind a recent spate of acts of mass violence, almost all of which have been perpetrated by men between the ages of 20 and 30.

"The preponderance of young men engaging in these deadly, evil, and stupid acts of violence may be a result of brains that have yet to fully developed," Howard Forman, an assistant professor of psychiatry at Albert Einstein College of Medicine, told my colleague Chris Weller.

Here are the milestones for brain maturity throughout life.

Age your brain matures at everything

SEE ALSO: The ages you’re the smartest at everything throughout your life

DON’T MISS: Scientists have found an exciting new clue about how ‘super-agers’ stay sharp as they age

Join the conversation about this story »

NOW WATCH: Scientists predict an unusually warm winter this year in most of the US — here’s why

from SAI http://read.bi/2m2kfkO

Warby Parker recommends glasses using your iPhone X’s depth camera


The depth-sensing front camera on the iPhone X isn’t just useful for unlocking your phone or making silly emoji clips. Eyewear maker Warby Parker has updated its Glasses app for iOS to include an iPhone X-only recommendation feature. Let the app scan your face and it’ll recommend the frames that are most likely to fit your measurements. This isn’t the same as modeling the frames on your face (wouldn’t the iPhone X be ideal for that?), but it could save you a lot of hemming and hawing as you wonder which styles are a good match.

This is something of a niche use — how often do you go shopping for frames, really? With that said, it illustrates how a depth-aware front cam can serve a genuinely practical purpose. If it can map your face and other objects in a scene, it can measure all kinds of data that just wouldn’t be an option with 2D photos. You might not want to buy an iPhone X just for this, but you may get more value out of it as developers get a feel for what TrueDepth can do.

Via: Joanna Stern (Twitter), The Verge

Source: App Store

from Engadget http://engt.co/2m3TqNg

CME Unveils Bitcoin Futures Circuit Breakers


Having taken a gamble on bitcoin futures, which are set to begin trading by the end of the year, the CME is now seeking to avoid the consequences of what has emerged as both the cryptocurrency’s best and worst selling point: its unprecedented volatility. To do that, the Chicago-based exchange will do what it does to virtually every other asset class traded under its roof, and impose limits on how much prices of bitcoin futures can fluctuate within a day.

While the CME already uses daily vol limits on most other markets, including crude, gold and market futures, to temporarily halt trading when price swings get out of
control, the CME has never before dealt with something like bitcoin, which in addition to being the world’s best performing asset classes in recent years, is also its most volatile. And, as the WSJ adds, it is also unclear how much impact CME’s limits will have on bitcoin, since its futures market has yet to emerge and most trading in the digital currency is on exchanges outside of CME’s control.

In any case, based on the CME’s preliminary term sheet, bitcoin trading limits would kick in when the price of its bitcoin futures move 7%, 13% or 20% up or down from the previous day’s closing price. The first two thresholds, for 7% and 13% moves, are “soft” limits, which would trigger a two-minute pause in trading of bitcoin futures. The 20% limit would be a “hard” stop on how far CME’s bitcoin futures could swing on any day.

By comparison, the CME has similar staggered volatility control on its popular E-mini S&P 500 futures contract, which also has three successive price-fluctuation limits at 7%, 13% and 20% during regular trading hours. A nighttime limit of 5% was hit in the S&P 500 futures on Nov. 8, 2016, when news of Donald Trump’s upset win in the U.S. presidential election triggered wild volatility in stock-market futures, only for the S&P to surge 21% in the 12 months since.

Of course, bitcoin will be a far "wilder" and more volatile instrument than the S&P 500 (one hopes). According to Coindesk calculations, so far in 2017 there have been two days in which bitcoin’s price swung more than 20% in a single day. There were 11 days in which it moved at least 13%, and 69 in which it moved at least 7%.

The full bitcoin contract specsheet is below.

from Zero Hedge http://bit.ly/2hTc5XD