On this month's Morbidly Fascinating Page:
A gene named after Sonic the Hedgehog was responsible for one-eyed sheep in the 1950s
TWO PHOTOS AT THE BOTTOM OF THIS PAGE COULD BE DISTUBING TO SOME.
In the Morbidly Fascinating Archives
Was Fox Mulder Right?
Strange Tombstones from Over the World
The Haunted Lemp Mansion
Radium Poisoning in the 1920s
The Suicide Forest
First: what happened? It began in the 1950s.
Idaho sheep ranchers couldn't figure out why, in the decade after World War II, a random batch of their lambs were being born with strange birth defects. The creatures had underdeveloped brains and a single eye planted, cyclopslike, in the middle of their foreheads. In 1957 they called in scientists from the U.S. Department of Agriculture to investigate.
The scientists worked for 11 years to solve the mystery. One of them, Lynn James, lived with the sheep for three summers before discovering the culprit: corn lilies. When the animals moved to higher ground during droughts, they snacked on the flowers. The lilies, it turned out, contained a poison, later dubbed cyclopamine, that stunted developing lamb embryos. The mothers remained unharmed. The case of the cyclopamine and the one-eyed Idaho lambs remained a freakish chemistry footnote for the next 25 years; researchers never could uncover why cyclopamine caused birth defects.
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What is the corn lily?
A corn lily is a perennial plant native to the western region of the United States. It grows under natural conditions in wetland areas, including meadows, creek beds, and marshes. Although several different species may be called by this name, it most often refers to the Veratrum californicum. The perennial is part of the the Liliaceae family. It is also poisonous, although it has been used for medicinal purposes.
This plant is known by several alternate names, all derived from its resemblance to other types of plants, including "Western hellebore" and "false hellebore." The Veratrum californicum is usually called a "corn lily" because its erect stalks and leaves resemble a cornstalk. The corn lily is also often mistaken for hellebore or skunk cabbage because of a superficial resemblance.
The alkaloids in Veratrum californicum render it poisonous to humans, animals, and some insects. The poison affects the nervous system, possibly resulting in paralysis, and may cause prolonged gestation and deformities in offspring.
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The positive outcome of the situation for cancer treatment
Now cancer researchers have improbably seized on the obscure plant chemical in corn lillies as the blueprint for a half-dozen promising tumor-fighters. Cyclopamine, it turns out, blocks the function of a gene called Sonic hedgehog that is essential for embryonic development but also plays a lead role in causing deadly cancers of the pancreas, skin, prostate and esophagus. "It's a beautiful accident of evolution," says Philip A. Beachy, a Howard Hughes Medical Institute investigator at Johns Hopkins University and one of the key players in this genetic detective story. In early tests researchers have stopped the growth of the most virulent human tumors, varieties accounting for 25% of cancer deaths.
Genentech and partner Curis, a fledgling biotech in Cambridge, Mass., are first out of the gate with an experimental hedgehog-blocking skin cream for basal cell skin cancer. More drugs in the pipeline are aimed at other cancers. Julian Adams, the superstar chemist who invented the AIDS drug Viramune and cancer drug Velcade, has made a hedgehog inhibitor one of his lead medicines at Infinity Pharmaceuticals. Little is known about his drug, which has not yet entered human trials, but on its Web site Infinity points to the role of hedgehog in pancreatic cancer. Novartis, Abbott Laboratories and Boehringer Ingelheim are all thought to be pursuing hedgehog programs. "It would be a major triumph for basic science if a drug were to come from this,"says Matthew Scott, an HHMI cancer genetics expert at Stanford University.
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Where does Sonic the Hedgehog come in?
Naming the varieties of hedgehog genes, which play a key role in embryo development, has been a source of friction among research teams. One group wanted to give the varieties numbers, another letters.
Dr. Clifford J. Tabin, a developmental biologist at Harvard Medical School, suggested they name each newly detected gene after a species of real hedgehogs. That is because the genes have a spiky appearance, resembling a hedgehog. This scheme stuck for the first three genes, which were designated Indian hedgehog, moonrat hedgehog and desert hedgehog.
But when Dr. Robert Riddle, a postdoctoral fellow working in Dr. Tabin's lab, detected what proved to be the most fascinating hedgehog gene of all, he rebeled against the system and decided to call the gene Sonic hedgehog, after a character in a Sega computer game. He pulled the name out of a comic book that his daughter brought over from the U.K. At the time, the videogame hadn't yet been released in the U.S. By the time it was, the paper detailing their process had already been published.
What is Cyclopia?
Cyclopia is usually a rare birth defect that occurs when the front part of the brain doesn’t cleave into right and left hemispheres.
The most obvious symptom of cyclopia is a single eye or a partially divided eye. A baby with cyclopia usually has no nose, but a proboscis (a nose-like growth) sometimes develops above the eye while the baby is in gestation.
Cyclopia, also known as alobar holoprosencephaly, occurs in about 1 in 100,000 births from natural causes, but when the corn lily flower is injested, one-eyed animals are more common.
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Are "cyclops" animals being born today?
The answer is yes, but mostly it occurs as a birth defect, not as an ingestion of the corn lily because that plant is only native to the Western United States.
The above goat was born in India in 2017.
Born on May 10, 2017, with a single, enormous eye in the center of its face, a baby goat has survived in a village in Assam, India.
The goat’s condition, called cyclopia, is characterized by the developing brain’s failure to separate into two hemispheres. As a result, the skull only forms a single eye socket.
Cyclopia is often accompanied by other physical deformities. This baby goat is also missing eyelashes, eyelids, one ear, and several teeth; its jaw is severely shortened, and its nose underdeveloped. Yet this particular goat has survived.
The owner, named Das, has said that the “miracle” goat has attracted dozens of daily visitors, some even trekking from neighboring villages.
“I believe this goat will bring luck to my home,” Das said.
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