Chapter 5. The Sensorimotor System

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By David Noonan The 63-year-old chief executive couldn't do his job. He had been crippled by migraine headaches throughout his adult life and was in the middle of a new string of attacks. “I have but a little moment in the morning in which I can either read, write or think,” he wrote to a friend. After that, he had to shut himself up in a dark room until night. So President Thomas Jefferson, in the early spring of 1807, during his second term in office, was incapacitated every afternoon by the most common neurological disability in the world. The co-author of the Declaration of Independence never vanquished what he called his “periodical head-ach,” although his attacks appear to have lessened after 1808. Two centuries later 36 million American migraine sufferers grapple with the pain the president felt. Like Jefferson, who often treated himself with a concoction brewed from tree bark that contained quinine, they try different therapies, ranging from heart drugs to yoga to herbal remedies. Their quest goes on because modern medicine, repeatedly baffled in attempts to find the cause of migraine, has struggled to provide reliable relief. Now a new chapter in the long and often curious history of migraine is being written. Neurologists believe they have identified a hypersensitive nerve system that triggers the pain and are in the final stages of testing medicines that soothe its overly active cells. These are the first ever drugs specifically designed to prevent the crippling headaches before they start, and they could be approved by the U.S. Food and Drug Administration next year. If they deliver on the promise they have shown in studies conducted so far, which have involved around 1,300 patients, millions of headaches may never happen. © 2015 Scientific American

Keyword: Pain & Touch
Link ID: 21662 - Posted: 11.28.2015

By Nala Rogers If you travel with a group of friends, you might delegate navigation to the person with the best sense of direction. But among homing pigeons, the leader is whoever flies the fastest—even if that pigeon has to pick up navigation skills on the job, according to a new study. To find out how the skills of individual pigeons influence flock direction, researchers tested four flocks on journeys from three different locations, each about 5 kilometers from their home loft near Oxford, U.K. At each site, the researchers tracked the pigeons during solo flights before releasing them together for several group journeys. The fastest birds surged to the front during group flights and determined when the flock turned, despite the fact that these leaders were often poor navigators during their initial solo expeditions. But on a final set of solo flights—made after the group journeys—these same leaders chose straighter routes than followers, the researchers report today in Current Biology. Apparently, being responsible for group decisions helped pigeons learn the route, say scientists, raising questions about the two-way interplay between skills and leadership. © 2015 American Association for the Advancement of Science

Keyword: Animal Migration; Learning & Memory
Link ID: 21661 - Posted: 11.28.2015

By Karen Weintraub Essential tremor is involuntary shaking – usually of the hands, but sometimes also of the neck, jaw, voice or legs. “Any fine tasks with the hands can be very difficult when the tremor is pronounced,” said Dr. Albert Hung, center director of the Massachusetts General Hospital National Parkinson Foundation Center of Excellence. Essential tremor can affect balance, walking, hearing and cognition, and can get worse over time, said Dr. Elan Louis, chief of the division of movement disorders at Yale School of Medicine. People with essential tremor run almost twice the risk of developing Alzheimer’s as the general population. Essential tremor appears with movement; if people let their hands sit still, they don’t tremble. That is the big difference between an essential tremor and the tremor of Parkinson’s disease, which can occur while at rest, Dr. Louis said. Essential tremor also tends to strike both hands while Parkinson’s is more one-sided at first, said Dr. Hung. The cause of essential tremor remains a mystery, though it seems to run in families. People of any age or sex can have the condition, though it is more common as people grow older. Roughly 4 percent of 40-year-olds have essential tremor, compared with about 20 percent of 90-year-olds, Dr. Louis said. Available treatments “aren’t great,” Dr. Louis said. Two medications – the beta blocker propranolol and the epilepsy drug primidone, sold under the brand name Mysoline – can reduce tremors by 10 to 30 percent, he said, but they work only in about half of patients. Deep brain stimulation – implanting electrodes into the brain to override faulty electrical signals – has been shown to markedly reduce hand tremor severity, he said. But the treatment can worsen cognitive and balance problems and “doesn’t cure the underlying disease. It merely and temporarily lessons a single symptom, which is the tremor.” © 2015 The New York Times Company

Keyword: Movement Disorders
Link ID: 21653 - Posted: 11.24.2015

By Gretchen Reynolds Sturdy legs could mean healthy brains, according to a new study of British twins. As I frequently have written in this column, exercise may cause robust improvements in brain health and slow age-related declines in memory and thinking. Study after study has shown correlations between physical activity, muscular health and mental acuity, even among people who are quite old. But these studies have limitations and one of them is that some people may be luckier than others. They may have been born to have a more robust brain than someone else. Their genes and early home environment might have influenced their brain health as much as or more than their exercise habits. Their genes and early home environment also might have influenced those exercise habits, as well as how their bodies and brains responded to exercise. In other words, genes and environment can seriously confound experimental results. That problem makes twins so valuable for scientific purposes. (Full disclosure, I am a twin, although not an identical one.) Twins typically share the same early home environment and many of the same genes, and if they are identical, all their genes are the same. So if one twin’s body, brain and thinking abilities begin to differ substantially over the years from their twin’s, the cause is less likely to be solely genetic or the early environment, and more likely to be attributable to lifestyle, including exercise habits. It was that possibility that recently prompted Claire Steves, a senior lecturer in twin research at King’s College London, to consider twins and their thighs. © 2015 The New York Times Company

Keyword: Alzheimers
Link ID: 21641 - Posted: 11.18.2015

Ian Sample Science editor Tiny biological compasses made from clumps of protein may help scores of animals, and potentially even humans, to find their way around, researchers say. Scientists discovered the minuscule magnetic field sensors in fruit flies, but found that the same protein structures appeared in retinal cells in pigeons’ eyes. They can also form in butterfly, rat, whale and human cells. The rod-like compasses align themselves with Earth’s geomagnetic field lines, leading researchers to propose that when they move, they act on neighbouring cell structures that feed information into the nervous system to create a broader direction-sensing system. Professor Can Xie, who led the work at Peking University, said the compass might serve as a “universal mechanism for animal magnetoreception,” referring to the ability of a range of animals from butterflies and lobsters to bats and birds, to navigate with help from Earth’s magnetic field. Whether the compasses have any bearing on human navigation is unknown, but the Peking team is investigating the possibility. “Human sense of direction is complicated,” said Xie. “However, I believe that magnetic sense plays a key role in explaining why some people have a good sense of direction.” The idea that animals could sense Earth’s magnetic field was once widely dismissed, but the ability is now well established, at least among some species. The greatest mystery that remains is how the sensing is done. © 2015 Guardian News and Media Limited

Keyword: Animal Migration
Link ID: 21638 - Posted: 11.17.2015

Ewen Callaway Ringo, a golden retriever born in 2003 in a Brazilian kennel, was never expected to live long. Researchers bred him and his littermates to inherit a gene mutation that causes severe muscular dystrophy. They hoped that the puppies would provide insight into Duchenne muscular dystrophy (DMD), an untreatable and ultimately fatal human disease caused by inactivation of the same gene. But Ringo’s muscles didn't waste away like his littermates', and researchers have now determined why: he was born with another mutation that seems to have protected him from the disease, according to a paper published in Cell1. Scientists hope that by studying Ringo’s mutation — which has never before been linked to muscular dystrophy — they can find new treatments for the disease. As many as 1 in 3,500 boys inherit mutations that produce a broken version of a protein called dystrophin, causing DMD. (The disease appears in boys because the dystrophin gene sits on the X chromosome, so girls must inherit two copies of the mutated gene to develop DMD.) The protein helps to hold muscle fibres together, and its absence disrupts the regenerative cycle that rebuilds muscle tissue. Eventually, fat and connective tissue replace muscle, and people with DMD often become reliant on a wheelchair before their teens. Few survive past their thirties. Some golden retriever females carry dystrophin mutations that cause a similar disease when passed onto male puppies. Dog breeders can prevent this through genetic screening. But Mayana Zatz, a geneticist at the University of São Paulo in Brazil, and her colleagues set out to breed puppies with the mutation to model the human disease. © 2015 Nature Publishing Group,

Keyword: Movement Disorders; Muscles
Link ID: 21632 - Posted: 11.14.2015

By Emily Underwood Researchers have found a way to increase how fast, and for how long, four paralyzed people can type using just their thoughts. The advance has to do with brain-machine interfaces (BCI), which are implanted in brain tissue and record hundreds of neurons firing as people imagine moving a computer cursor. The devices then use a computer algorithm to decode those signals and direct a real cursor toward words and letters on a computer screen. One of the biggest problems with BCIs is the brain itself: When the soft, squishy organ shifts in the skull, as it frequently does, it can displace the electrode implants. As a result, the movement signal extracted from neuronal firing is constantly being distorted, making it impossible for a patient to keep the cursor from drifting off course without a researcher recalibrating the instrument every 10 minutes or so. In the new study, part of a clinical trial of BCIs called BrainGate, researchers performed several software tweaks that allow the devices to self-correct in real time by calculating the writer’s intention based on the words they’ve already written. The devices can now also correct for neuronal background noise whenever a person stops typing. These improvements, demonstrated in the video above, allow BCI users to type faster and for longer periods of time, up to hours or days, the team reports today in Science Translational Medicine. Though the technology still needs to be miniaturized and wireless before it can be used outside of the lab, the new work is a big step towards BCIs that paralyzed people can use on their own at home, the scientists say. © 2015 American Association for the Advancement of Science

Keyword: Robotics
Link ID: 21626 - Posted: 11.12.2015

By Virginia Morell Plunge a live crab into a pot of boiling water, and it’s likely to try to scramble out. Is the crab’s behavior simply a reflex, or is it a sign of pain? Many scientists doubt that any invertebrate (or fish) feels pain because they lack the areas in the brain associated with human pain. Others argue this is an unfair comparison, noting that despite the major differences between vertebrate and invertebrate brains, their functions (such as seeing) are much the same. To get around this problem, researchers in 2014 argued that an animal could be classified as experiencing pain if, among other things, it changes its behavior in a way that indicates it’s trying to prevent further injury, such as through increased wariness, and if it shows a physiological change, such as elevated stress hormones. To find out whether crabs meet these criteria, scientists collected 40 European shore crabs (Carcinus maenas), shown in the photo above, in Northern Ireland. They placed the animals into individual tanks, and gave half 200-millisecond electrical shocks every 10 seconds for 2 minutes in their right and left legs. The other 20 crabs served as controls. Sixteen of the shocked crabs began walking in their tanks, and four tried to climb out. None of the control crabs attempted to clamber up the walls, but 14 walked, whereas six didn’t move at all. There was, however, one big physiological difference between the 16 shocked, walking crabs and the 14 control walkers, the scientists report in today’s issue of Biology Letters: Those that received electrical jolts had almost three times the amount of lactic acid in their haemolymph, a fluid that’s analogous to the blood of vertebrates—a clear sign of stress. Thus, crabs pass the bar scientists set for showing that an animal feels pain. © 2015 American Association for the Advancement of Science.

Keyword: Pain & Touch; Evolution
Link ID: 21623 - Posted: 11.11.2015

By Arlene Karidis Several years ago, Peggy Chenoweth began having excruciating cramping in her ankle. It felt severely sprained and as if her toe were twisting to the point where it was being ripped off her foot. “The pain is right here,” she told an orthopedic surgeon, “in my ankle and foot.” But the 41-year-old Gainesville, Va., resident no longer had that ankle and foot. Her leg had been amputated below the knee after a large piece of computer equipment fell off a cart, crushed her foot and caused nerve damage. Further, she insisted that since the amputation, she could feel her missing toes move. Chenoweth’s surgeon knew exactly what was going on: phantom pain. Lynn Webster, an anesthesiologist and past president of the American Academy of Pain Medicine, explains the phenomenon: “With ‘phantom pain,’ nerves that transmitted information from the brain to the now-missing body part continue to send impulses, which relay the message of pain.” It feels as if the removed part is still there and hurting, but pain is actually in the brain. The sensation ranges from annoying itching to red-hot burning. Physicians wrote about phantom pain as early as the 1860s, but U.S. research on this condition has increased recently, spurred by the surge of amputees returning from warfare in Iraq and Afghanistan and by increasing rates of diabetes. (Since 2003, nearly 1,650 service members have lost limbs, according to the Congressional Research Service. In 2010, about 73,000 amputations were performed on diabetics in the United States, according to the Centers for Disease Control and Prevention.)

Keyword: Pain & Touch
Link ID: 21620 - Posted: 11.10.2015

By Jason G. Goldman When a monkey has the sniffles or a headache, it doesn't have the luxury of popping a few painkillers from the medicine cabinet. So how does it deal with the common colds and coughs of the wildlife world? University of Georgia ecologist Ria R. Ghai and her colleagues observed a troop of more than 100 red colobus monkeys in Uganda's Kibale National Park for four years to figure out whether the rain forest provides a Tylenol equivalent. Monkeys infected with a whipworm parasite were found to spend more time resting and less time moving, grooming and having sex. The infected monkeys also ate twice as much tree bark as their healthy counterparts even though they kept the same feeding schedules. The findings were published in September in the journal Proceedings of the Royal Society B. The fibrous snack could help literally sweep the intestinal intruder out of the simians' gastrointestinal tracts, but Ghai suspects a more convincing reason. Seven of the nine species of trees and shrubs preferred by sick monkeys have known pharmacological properties, such as antisepsis and analgesia. Thus, the monkeys could have been self-medicating, although she cannot rule out other possibilities. The sick individuals were, however, using the very same plants that local people use to treat illnesses, including infection by whipworm parasites. And that “just doesn't seem like a coincidence,” Ghai says. © 2015 Scientific American,

Keyword: Drug Abuse; Evolution
Link ID: 21619 - Posted: 11.10.2015

by Laura Sanders Babies’ minds are mysterious. Thoughts might be totally different in a brain that lacks words, and sensations might feel alien in a body so new. Are babies’ perceptions like ours, or are they completely different? Even if babies could talk, words would surely fail to convey what it’s like to experience, oh, every single thing for the first time. A recent paper offers a sliver of insight into young babies’ inner lives. The study, published October 19 in Current Biology, finds an example in which 4-month-old babies are happily oblivious to the external world. The research focuses on a perceptual trick that suckers adults and 6-month-old babies alike. When the hands are crossed, people often mistake which hand feels a touch. Let’s say your left hand (now crossed over to the right side of your body) gets a tickle. Your eyes would see a hand on the right side of your body get touched — a place usually claimed by your right hand, but now occupied by your left. Those mismatches between sight, touch and expectation can thwart you from quickly and correctly saying which hand was touched. Here’s the twist: 4-month-old babies don’t fall for this trick, Andrew Bremner of Goldsmiths, University of London and his colleagues found. In the experiment, a researcher would hold infants’ legs in either a crossed position or straight, while one of two remote-controlled buzzers taped to their feet tickled one foot. The researchers then watched which foot or leg wiggled as a result. If the buzzed foot moved, that meant that the baby got it right. © Society for Science & the Public 2000 - 2015.

Keyword: Development of the Brain; Pain & Touch
Link ID: 21613 - Posted: 11.07.2015

David Cyranoski A Chinese neuroscientist has been sacked after reporting he had used magnetic fields to control neurons and muscle cells in nematode worms (pictured), using a protein that senses magnetism. Tsinghua University in Beijing has sacked a neuroscientist embroiled in a dispute over work on a long-sought protein that can sense magnetic fields. The university has not given a specific reason for its dismissal, however, and the scientist involved, Zhang Sheng-jia, says that he will contest their action. In September, Zhang reported in the journal Science Bulletin1 that he could manipulate neurons in worms by applying a magnetic field — a process that uses a magnetic-sensing protein. But a biophysicist at neighbouring Peking University, Xie Can, who claims to have discovered the protein’s magnetic-sensing capacity and to have a paper detailing his research under review, complained that Zhang should not have published his paper before Xie’s own work appeared. Xie said that by publishing, Zhang violated an agreement that the pair had reached — although the two scientists tell different versions about the terms of their agreement, and have different explanations of how Zhang came to be working with the protein. © 2015 Nature Publishing Group

Keyword: Animal Migration
Link ID: 21608 - Posted: 11.06.2015

By SINDYA N. BHANOO Some kinds of itching can be caused by the lightest of touches, a barely felt graze that rustles tiny hairs on the skin’s surface. This type of itch is created via a dedicated neural pathway, a new study suggests. The finding, which appears in the journal Science, could help researchers better understand chronic itchiness in conditions like eczema, diabetic neuropathy, multiple sclerosis and some cancers. The study also may help researchers determine why certain patients do not respond well to antihistamine drugs. “In the future, we may have some way to manipulate neuron activity to inhibit itching,” said Quifu Ma, a neurobiologist at Harvard University and one of the study’s authors. In the study, Dr. Ma and his colleagues inhibited neurons that express a neuropeptide known as Y or NPY in mice. When these neurons were suppressed and the mice were poked with a tiny filament, they fell into scratching fits. Normally, mice would not even respond to this sort of stimuli. “We start to see skin lesions — they don’t stop scratching,” Dr. Ma said. “It’s pretty traumatic.” The neurons only seem related to itches prompted by light touching, known as mechanically induced itches. Chemical itches, like those caused by a mosquito bite or an allergic reaction, are not transmitted by the same neurons. © 2015 The New York Times Company

Keyword: Pain & Touch
Link ID: 21593 - Posted: 11.03.2015

By Hanae Armitage Fake fingerprints might sound like just another ploy to fool the feds. But the world’s first artificial prints—reported today—have even cooler applications. The electronic material, which mimics the swirling designs imprinted on every finger, can sense pressure, temperature, and even sound. Though the technology has yet to be tested outside the lab, researchers say it could be key to adding sensation to artificial limbs or even enhancing the senses we already have. “It’s an interesting piece of work,” says John Rogers, materials scientist at the University of Illinois, Urbana-Champaign, who was not involved in the study. “It really adds to the toolbox of sensor types that can be integrated with the skin.” Electronic skins, known as e-skins, have been in development for years. There are several technologies used to mimic the sensations of real human skin, including sensors that can monitor health factors like pulse or temperature. But previous e-skins have been able to “feel” only two sensations: temperature and pressure. And there are additional challenges when it comes to replicating fingertips, especially when it comes to mimicking their ability to sense even miniscule changes in texture, says Hyunhyub Ko, a chemical engineer at Ulsan National Institute of Science and Technology in South Korea. So in the new study, Ko and colleagues started with a thin, flexible material with ridges and grooves much like natural fingerprints. This allowed them to create what they call a “microstructured ferroelectric skin” The e-skin’s perception of pressure, texture, and temperature all come from a highly sensitive structure called an interlocked microdome array—the tiny domes sandwiched in the bottom two layers of the e-skin, also shown in the figure below. © 2015 American Association for the Advancement of Science

Keyword: Pain & Touch; Robotics
Link ID: 21588 - Posted: 10.31.2015

Laura Sanders A fly tickling your arm hair can spark a maddening itch. Now, scientists have spotted nerve cells in mice that curb this light twiddling sensation. If humans possess similar itch-busters, the results, published in the Oct. 30 Science, could lead to treatments for the millions of people who suffer from intractable, chronic itch. For many of these people, there are currently no good options. “This is a major problem,” says clinician Gil Yosipovitch of Temple University School of Medicine in Philadelphia and director of the Temple Itch Center. The new study shows that mice handle an itch caused by a fluttery touch differently than other kinds of itch. This distinction “seems to have clinical applications that clearly open our field,” Yosipovitch says. In recent years, scientists have made progress teasing apart the pathways that carry itchy signals from skin to spinal cord to brain (SN: 11/22/2008, p. 16). But those itch signals often originate from chemicals, such as those delivered by mosquitoes. All that’s needed to spark a different sort of itch, called mechanical itch, is a light touch on the skin. The existence of this kind of itch is no surprise, Yosipovitch says. Mechanical itch may help explain why clothes or even dry, scaly skin can be itchy. The new finding came from itchy mice engineered to lack a type of nerve cell in their spinal cords. Without prompting, these mice scratched so often that they developed sore bald patches on their skin. © Society for Science & the Public 2000 - 2015

Keyword: Pain & Touch
Link ID: 21587 - Posted: 10.31.2015

Adam Cole Watch a scary movie and your skin crawls. Goose bumps have become so associated with fear that the word is synonymous with thrills and chills. But what on earth does scary have do to with chicken-skin bumps? For a long time, it wasn't well understood. Physiologically, it's fairly simple. Adrenaline stimulates tiny muscles to pull on the roots of our hairs, making them stand out from our skin. That distorts the skin, causing bumps to form. Call it horripilation, and you'll be right — bristling from cold or fear. Charles Darwin once investigated goose bumps by scaring zoo animals with a stuffed snake. He argued for the now accepted theory that goose bumps are a vestige of humanity's ancient past. Our ancestors were hairy. Goose bumps would have fluffed up their hair. When they were scared, that would have made them look bigger — and more intimidating to attackers. When they were cold, that would have trapped an insulating layer of air to keep them warm. We modern humans still get goose bumps when we're scared or cold, even though we've lost the advantage of looking scarier or staying warmer ourselves. And researchers have found that listening to classical music (or Phil Collins), seeing pictures of children or drinking a sour drink can also inspire goose bumps. There's clearly a link with emotion and reward, too. © 2015 npr

Keyword: Pain & Touch; Emotions
Link ID: 21586 - Posted: 10.31.2015

By Diana Kwon Six years before her husband was diagnosed with Parkinson’s disease, a progressive neurodegenerative disorder marked by tremors and movement difficulties, Joy Milne detected a change in his scent. She later linked the subtle, musky odor to the disease when she joined the charity Parkinson’s UK and met others with the same, distinct smell. Being one of the most common age-related disorders, Parkinson’s affects an estimated seven million to 10 million people worldwide. Although there is currently no definitive diagnostic test, researchers hope that this newly found olfactory signature will lead help create one. Milne, a super-smeller from Perth, Scotland, wanted to share her ability with researchers. So when Tilo Kunath, a neuroscientist at the University of Edinburgh, gave a talk during a Parkinson’s UK event in 2012, she raised her hand during the Q&A session and claimed she was able to smell the disease. “I didn’t take her seriously at first,” Kunath says. “I said, ‘No, I never heard of that, next question please.’” But months later Kunath shared this anecdote with a colleague and received a surprising response. “She told me that that lady wasn’t wrong and that I should find her,” Kunath says. Once the researchers found Milne, they tested her claim by having her sniff 12 T-shirts: six that belonged to people with Parkinson’s and six from healthy individuals. Milne correctly identified 11 out of 12, but miscategorized one of the non-Parkinson’s T-shirts in the disease category. It turned out, however, she was not wrong at all—that person would be diagnosed with Parkinson’s less than a year later. © 2015 Scientific American

Keyword: Parkinsons; Chemical Senses (Smell & Taste)
Link ID: 21582 - Posted: 10.31.2015

By Diana Kwon | In the human form of mad cow disease, called Creutzfeldt-Jakob, a person's brain deteriorates—literally developing holes that cause rapidly progressing dementia. The condition is fatal within one year in 90 percent of cases. The culprits behind the disease are prions—misfolded proteins that can induce normal proteins around them to also misfold and accumulate. Scientists have known that these self-propagating, pathological proteins cause some rare brain disorders, such as kuru in Papua New Guinea. But growing evidence suggests that prions are at play in many, if not all, neurodegenerative disorders, including Alzheimer's, Huntington's and Parkinson's, also marked by aggregations of malformed proteins. Until recently, there was no evidence that the abnormal proteins found in people who suffer from these well-known diseases could be transmitted directly from person to person. The tenor of that discussion suddenly changed this September when newly published research in the journal Nature provided the first hint such human-to-human transmission may be possible. (Scientific American is part of Springer Nature.) For the study, John Collinge, a neurologist at University College London, and his colleagues conducted autopsies on eight patients who died between the ages of 36 and 51 from Creutzfeldt-Jakob. All the subjects had acquired the disease after treatment with growth hormone later found to be contaminated with prions. The surprise came when the researchers discovered that six of the brains also bore telltale signs of Alzheimer's—in the form of clumps of beta-amyloid proteins, diagnostic for the disease—even though the patients should have been too young to exhibit such symptoms. © 2015 Scientific American,

Keyword: Prions; Alzheimers
Link ID: 21580 - Posted: 10.29.2015

by Helen Thompson Five, six, seven, eight! All together now, let's spread those jazz hands and get moving, because synchronized dancing improves our tolerance of pain and helps us bond as humans, researchers suggest October 28 in Biology Letters. A team of psychologists at the University of Oxford taught high school students varied dance routines — each requiring different levels of exertion and synchronized movement — and then tested their pain tolerance with the sharp squeeze of a blood pressure cuff. Statistically, routines with more coordinated choreography and full body movement produced higher pain thresholds and sunny attitudes toward others in the group. Coordinated dancing with a group and exerting more energy may independently promote the release of pain-blocking endorphins as well as increase social bonding, the team writes. |© Society for Science & the Public 2000 - 2015

Keyword: Pain & Touch
Link ID: 21575 - Posted: 10.28.2015

By Hanae Armitage CHICAGO, ILLINOIS—Huntingtons disease, a neurological condition caused by brain-destroying mutant proteins, starts with mood swings and twitching and ends in dementia and death. The condition, which afflicts about 30,000 Americans, has no cure. But now, a new gene-editing method that many believe will lead to a Nobel Prize has been shown to effectively halt production of the defective proteins in mice, leading to hope that a potent therapy for Huntingtons is on the distant horizon. That new method is CRISPR, which uses RNA-guided enzymes to snip out or add segments of DNA to a cell. In the first time it has been applied to Huntingtons disease, CRISPR’s results are “remarkably encouraging,” says neuroscientist Nicole Déglon of the University of Lausanne in Switzerland, who led the mouse study, results of which she and her co-researcher Nicolas Merienne shared yesterday at the Society for Neuroscience Conference in Chicago, Illinois. As neurological diseases go, Huntingtons is an ideal candidate for CRISPR therapy, because the disease is determined by a single gene, Déglon notes. A mutation in the gene, which codes for a normally helpful brain protein called huntingtin, consists of different numbers of “tandem repeats,” repeating segments of DNA that cause the protein to fold into a shape that is toxic to the brain. Déglon and her team wondered whether CRISPR could halt production of this dangerous molecule. Using a virus as a delivery vehicle, the researchers infected two separate groups of healthy adult mice with a mutant huntingtin gene, but only one group received the therapy: a CRISPR “cassette,” which includes DNA for the gene-editing enzyme Cas9 and the RNA to target the huntingtin gene. © 2015 American Association for the Advancement of Science

Keyword: Huntingtons
Link ID: 21538 - Posted: 10.21.2015