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Why the Fugate Family Has Blue Skin

ABCNews
ABCNews

No need to adjust the colors on your monitor, or spend time trying to figure out how the image above was altered. It wasn't.

The family pictured above, the Fugates of eastern Kentucky, actually have blue-tinged skin, the result of a condition called methemoglobinemia. It’s caused by a type of hemoglobin that can’t carry oxygen through the blood—and because the blood isn’t oxygenated, it makes skin look blue, lips look purple, and blood look chocolate-colored.

Most people have less than 1 percent of methemoglobin. When that level rises to 10-20 percent, the result is blue-tinged skin. While the genetic condition didn’t seem to have any notable health impact, it did affect the Fugate family psychologically, causing them to retreat from public life.

They also had to deal with the shame associated with inbreeding. Think back to the Punnett squares you had to diagram in 8th-grade science class. The gene that creates methemoglobinemia is recessive, so it likely wouldn’t have been a problem—except that the Fugate family married within itself. This wasn’t uncommon back in the 1800s, when cousins married cousins without a second thought. But as more became known about genetics and the consequences of reproducing with family members, the Fugates’ blue skin became almost like a scarlet letter … or an indigo letter, as it were. And so they withdrew further from society, becoming something of an urban legend in Kentucky.

In the 1960s, Dr. Madison Cawein was able to test two Fugate descendants, Patrick and Rachel Ritchie. “They were bluer’n hell,” Cawein said. “They were really embarrassed about being blue. They wouldn’t come into the waiting room. You could tell how much it bothered them.” After conducting many blood tests, Cawein came to the conclusion that methemoglobinemia was the root of the problem, and determined that he could convert the methemoglobin back by simply injecting a missing enzyme to oxygenate the blood. Strangely, the easiest way to do this was with a chemical called methylene blue. Though the family thought the doctor was completely crazy—after all, how could injecting them with another blue substance make them less blue?—they allowed Cawein to give it a shot.

It worked almost instantaneously. ''Within a few minutes, the blue color was gone from their skin," the doctor said. "For the first time in their lives, they were pink. They were delighted."

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Courtesy of Nature
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science
Scientists Create Three Puppy Clones of 'Snuppy,' the World's First Cloned Dog
Courtesy of Nature
Courtesy of Nature

Snuppy, the world's first cloned dog, died in 2015, but his genetic legacy lives on. As the National Post reports, South Korean scientists recently described in the journal Scientific Reports the birth of three clone puppies, all of which are identical replicas of the famous Afghan hound.

Those who lived through the 1990s might remember Dolly, the Scottish sheep that gained fame for being the very first mammal to be cloned from an adult cell. Following Dolly's 1996 cloning, scientists managed to replicate other animals, including cats, mice, cows, and horses. But dog cloning initially stymied scientists, Time reports, as their breeding period is limited and their eggs are also hard to extract.

Ultimately, researchers ended up using somatic cell nuclear transfer (SCNT) to clone a dog, the same method that was used to make Dolly. In the early 2000s, a team of South Korean scientists inserted DNA harvested from an Afghan hound's skin cells into a dog egg from which the DNA had been removed. The egg divided, which produced multiple cloned embryos.

The scientists implanted 1095 of these embryos in 123 dogs, an exhaustive initiative that yielded just three pregnancies, according to NPR. Of these, Snuppy—whose name is a combination of "puppy" and Seoul National University's initials—was the only survivor.

Snuppy died from cancer in April 2015, just shortly after his 10th birthday. To celebrate his successful life, the same South Korean researchers decided to re-clone him using mesenchymal stem cells from the dog's belly fat, which were taken when he was five. This time around, they transferred 94 reconstructed embryos to seven dogs. Four clones were later born, although one ended up dying shortly after birth.

The tiny Snuppy clones are now more than a year old, and researchers say that they don't think that the pups face the risk of accelerated aging, nor are they more disease-prone than other dogs. (Dolly died when she was just six years old, while cloned mice have also experienced shorter lifespans.) Snuppy's somatic cell donor, Tai, lived just two years longer than Snuppy, dying at age 12, the average lifespan of an Afghan hound.

Researchers say that this new generation of Snuppys will yield new insights into the health and longevity of cloned animals. Meanwhile, in other animal cloning news, a Texas-based company called ViaGen Pets is now offering to clone people's beloved pets, according to CBS Pittsburgh—a service that costs a cool $50,000 for dogs.

[h/t National Post]

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Food
Researchers Pinpoint the Genes Behind the Durian's Foul Stench
iStock
iStock

Durian is a popular fruit in parts of southeast Asia. It's also known for having the most putrid, off-putting odor of any item sold in the produce section. Even fans of durian know why the fruit gets a bad rap, but what exactly causes its divisive scent is less obvious. Determined to find the answer, a team of researchers funded by "a group of anonymous durian lovers" mapped the fruit's genome, as reported by the BBC.

The study, published in the journal Nature Genetics [PDF], contains data from the first-ever complete genetic mapping of a durian fruit. It confirms that durian's excess stinkiness comes from sulfur, a chemical element whose scent is often compared to that of rotten eggs.

Analysis of the fruit's chemical makeup has been done in the past, so the idea that sulfur is a major contributor to its signature smell is nothing new. What is new is the identification of the specific class of sulfur-producing genes. These genes pump out sulfur at a "turbocharged" rate, which explains why the stench is powerful enough to have durian banned in some public areas. It may seem like the smell is a defense mechanism to ward off predators, but the study authors write that it's meant to have the opposite effect. According to the paper, "it is possible that linking odor and ripening may provide an evolutionary advantage for durian in facilitating fruit dispersal." In other words, the scent attracts hungry primates that help spread the seeds of ripe durian fruits by consuming them.

The revelation opens the door to genetically modified durian that are tweaked to produce less sulfur and therefore have a milder taste and smell. But such a product would likely inspire outrage from the food's passionate fans. While the flavor profile has been compared to rotten garbage and dead animal meat, it's also been praised for its "overtones of hazelnut, apricot, caramelized banana, and egg custard" by those who appreciate its unique character.

[h/t BBC]

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