Chapter 11. Motor Control and Plasticity
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By Simon Makin Scientists claim to have discovered the first new human prion in almost 50 years. Prions are misfolded proteins that make copies of themselves by inducing others to misfold. By so doing, they multiply and cause disease. The resulting illness in this case is multiple system atrophy (MSA), a neurodegenerative disease similar to Parkinson's. The study, published August 31 in Proceedings of the National Academy of Sciences, adds weight to the idea that many neurodegenerative diseases are caused by prions. In the 1960s researchers led by Carleton Gajdusek at the National Institutes of Health transmitted kuru, a rare neurodegenerative disease found in Papua New Guinea, and Creutzfeldt–Jakob disease (CJD), a rare human dementia, to chimpanzees by injecting samples from victims' brains directly into those of chimps. It wasn't until 1982, however, that Stanley Prusiner coined the term prion (for “proteinaceous infectious particle”) to describe the self-propagating protein responsible. Prusiner and colleagues at the University of California, San Francisco, showed this process caused a whole class of diseases, called spongiform encephalopathies (for the spongelike appearance of affected brains), including the bovine form known as “mad cow” disease. The same protein, PrP, is also responsible for kuru, which was spread by cannibalism; variant-CJD, which over 200 people developed after eating beef infected with the bovine variety; and others. The idea that a protein could transmit disease was radical at the time but the work eventually earned Prusiner the 1997 Nobel Prize in Physiology or Medicine. He has long argued prions may underlie other neurodegenerative diseases but the idea has been slow to gain acceptance. © 2015 Scientific American
Boer Deng Palaeontologist Stephen Gatesy wants to bring extinct creatures to life — virtually speaking. When he pores over the fossilized skeletons of dinosaurs and other long-dead beasts, he tries to imagine how they walked, ran or flew, and how those movements evolved into the gaits of their modern descendents. “I'm a very visual guy,” he says. But fossils are lifeless and static, and can only tell Gatesy so much. So instead, he relies on XROMM, a software package that he developed with his colleagues at Brown University in Providence, Rhode Island. XROMM (X-ray Reconstruction of Moving Morphology) borrows from the technology of motion capture, in which multiple cameras film a moving object from different angles, and markers on the object are rendered into 3D by a computer program. The difference is that XROMM uses not cameras, but X-ray machines that make videos of bones and joints moving inside live creatures such as pigs, ducks and fish. Understanding how the movements relate to the animals' bone structure can help palaeontologists to determine what movements would have been possible for fossilized creatures. “It's a completely different approach” to studying evolution, says Gatesy. XROMM, released to the public in 2008 as an open-source package, is one of a number of software tools that are expanding what researchers know about how animals and humans walk, crawl and, in some cases, fly (see ‘Movement from inside and out’). That has given the centuries-old science of animal motion relevance to a wide range of fields, from studying biodiversity to designing leg braces, prostheses and other assistive medical devices.“We're in an intense period of using camera-based and computer-based approaches to expand the questions we can ask about motion,” says Michael Dickinson, a neuroscientist at the California Institute of Technology in Pasadena. © 2015 Nature Publishing Group
Keyword: Movement Disorders
Link ID: 21370 - Posted: 09.01.2015
By Diana Kwon Each year doctors diagnose approximately 60,000 Americans with Parkinson’s disease, an incurable neurodegenerative condition for which the number-one risk factor is age. Worldwide an estimated seven to 10 million people currently live with the malady. As U.S. and global populations grow older, it is becoming increasingly urgent to understand its causes. So far, researchers know that Parkinson’s involves cell death in a few restricted areas of the brain including the substantia nigra (SNc), one of two big cell clusters in the midbrain that house a large population of dopamine neurons. These cells release dopamine and are involved in a variety of functions including reward processing and voluntary movement. Their death leads to the motor control and balance issues that are core symptoms of the disease. New research shows that these brain cells, most at risk in Parkinson’s disease, require unusually high amounts of energy to carry out their tasks because of their highly branched structures. Like a massive car with an overheating engine, these neurons are susceptible to burnout and early death. This discovery emerged from a comparison of energy use in nigral dopamine neurons and in similar neurons found in the nearby ventral tegmental area (VTA), also in the midbrain. “We were trying to understand why dopamine neurons of the substantia nigra die in Parkinson’s disease patients while there are so many other brain cells that have no problem at all,” says Louis-Eric Trudeau, a neuroscientist at the University of Montreal and senior author of the study published in the August 27 Current Biology. © 2015 Scientific American,
Link ID: 21353 - Posted: 08.28.2015
By Emily Underwood It is famous for robbing Lou Gehrig of his life and Stephen Hawking of his mobility and voice, but just how amyotrophic lateral sclerosis (ALS) destroys motor neurons in the brain and spinal cord remains a mystery. Now, scientists are converging on an explanation, at least for a fraction of the ALS cases caused by a specific mutation. In cells with the mutation, the new work shows, pores in the membrane separating the nucleus and cytoplasm become clogged, preventing vital molecules from passing through and creating a fatal cellular traffic jam. For now, the work applies only to the mutation dubbed C9orf72—a DNA stutter in which a short nucleotide sequence, GGGGCC, is repeated hundreds to thousands of times in a gene on chromosome 9. Nor do the multiple labs reporting results this week agree on exactly what plugs those nuclear pores and how the cells die. Still, the work is “a major breakthrough” in ALS research, says Amelie Gubitz, program director of the neurodegeneration division at the National Institute of Neurological Disorders in Bethesda, Maryland. The groups worked independently, starting with different hypotheses and experimental designs, yet reached similar conclusions, making the finding more convincing. And it suggests that boosting traffic through nuclear pores could be a new strategy for treating some cases of ALS and frontotemporal dementia (FTD), another neurodegenerative condition C9orf72 can cause. Based on past work by their own and other groups, neuroscientists Jeff Rothstein and Tom Lloyd at Johns Hopkins University in Baltimore, Maryland, suspected that the long strands of excess RNA produced by C9orf72 cause neurodegeneration by binding to, and thus sequestering, key cellular proteins. The team tested the idea in fruit flies with the mutation, which display damage in the nerve cells of their eyes and in motor neurons. © 2015 American Association for the Advancement of Science
A healthy motor neuron needs to transport its damaged components from the nerve-muscle connection all the way back to the cell body in the spinal cord. If it cannot, the defective components pile up and the cell becomes sick and dies. Researchers at the National Institutes of Health’s National Institute of Neurological Disorders and Stroke (NINDS) have learned how a mutation in the gene for superoxide dismutase 1 (SOD1), which causes ALS, leads cells to accumulate damaged materials. The study, published in the journal Neuron, suggests a potential target for treating this familial form of ALS. More than 12,000 Americans have ALS, also known as Lou Gehrig’s disease, and roughly 5-10 percent of them inherited a genetic mutation from a parent. These cases of familial ALS are often caused by mutations in the gene that codes for SOD1, an important enzyme located in the neuron’s mitochondria, the cell’s energy-producing structures. This mutation causes the death of motor neurons that control the patient’s muscles, resulting in progressive paralysis. “About 90 percent of the energy in the brain is generated by mitochondria,” said Zu-Hang Sheng, Ph.D., an NINDS scientist and the study’s senior author. “If the mitochondria aren’t healthy, they produce energy less efficiently; they can also release harmful chemicals called reactive oxygen species that cause cell death. As a consequence, mitochondrial damage can cause neurodegeneration.” In healthy neurons, storage containers called late endosomes collect damaged mitochondria and various destructive chemicals. A motor protein called dynein then transports the endosomes to structures called lysosomes, which use the chemicals to break down the endosomes. Dr. Sheng’s team discovered that this crucial process is faulty in nerve cells with SOD1 mutations because mutant SOD1 interferes with a critical molecule called snapin that hooks the endosome to the dynein motor protein.
Keyword: ALS-Lou Gehrig's Disease
Link ID: 21294 - Posted: 08.13.2015
Sarah Schwartz In 2011, science journalist Jon Palfreman saw a doctor about a tremor in his left hand. The doctor diagnosed Palfreman, then 60, with Parkinson’s disease. The disorder, which is newly diagnosed in 60,000 Americans each year, promised a crippling future of tremors, loss of mobility, dementia and more. Palfreman decided to use his reporting expertise to investigate how Parkinson’s disease affects the body and learn about efforts to find a cure. With Brain Storms, Palfreman follows Parkinson’s history from the careful observations of 19th century physicians to today’s cutting-edge research. Palfreman relates complex research studies as gripping medical mysteries. He describes how scientists connected Parkinson’s with the dramatic loss of the brain chemical dopamine and with tenacious protein knots called Lewy bodies that are a hallmark of the disease. Palfreman also explores treatments past and present, including the widely used drug levodopa that restores motion (sometimes uncontrollably), gene therapies, brain surgeries and promising experimental antibody treatments that attack and dissolve misfolded Parkinson’s-related proteins. Ultimately, Brain Storms is about more than Parkinson’s disease; it’s about the people living with the disorder. Palfreman describes patients who must teach themselves to walk without falling over or who freeze in place. He writes about a researcher driven to search for a cure after the disease affects his own father. © Society for Science & the Public 2000 - 2015
Link ID: 21284 - Posted: 08.12.2015
By David Noonan Leaping through the air with ease and spinning in place like tops, ballet dancers are visions of the human body in action at its most spectacular and controlled. Their brains, too, appear to be special, able to evade the dizziness that normally would result from rapid pirouettes. When compared with ordinary people's brains, researchers found in a study published early this year, parts of dancers' brains involved in the perception of spinning seem less sensitive, which may help them resist vertigo. For millions of other people, it is their whole world, not themselves, that suddenly starts to whirl. Even the simplest task, like walking across the room, may become impossible when vertigo strikes, and the condition can last for months or years. Thirty-five percent of adults older than 39 in the U.S.—69 million people—experience vertigo at one time or another, often because of damage to parts of the inner ear that sense the body's position or to the nerve that transmits that information to the brain. Whereas drugs and physical therapy can help many, tens of thousands of people do not benefit from existing treatments. “Our patients with severe loss of balance have been told over and over again that there's nothing we can do for you,” says Charles Della Santina, an otolaryngologist who studies inner ear disorders and directs the Johns Hopkins Vestibular NeuroEngineering Laboratory. Steve Bach's nightmare started in November 2013. The construction manager was at home in Parsippany, N.J. “All of a sudden the room was whipping around like a 78 record,” says Bach, now age 57. He was curled up on the living room floor in a fetal position when his daughter found him and called 911. He spent the next five days in the hospital. © 2015 Scientific American
Keyword: Movement Disorders
Link ID: 21248 - Posted: 08.01.2015
Five men with complete motor paralysis were able to voluntarily generate step-like movements thanks to a new strategy that non-invasively delivers electrical stimulation to their spinal cords, according to a new study funded in part by the National Institutes of Health. The strategy, called transcutaneous stimulation, delivers electrical current to the spinal cord by way of electrodes strategically placed on the skin of the lower back. This expands to nine the number of completely paralyzed individuals who have achieved voluntary movement while receiving spinal stimulation, though this is the first time the stimulation was delivered non-invasively. Previously it was delivered via an electrical stimulation device surgically implanted on the spinal cord. In the study, the men’s movements occurred while their legs were suspended in braces that hung from the ceiling, allowing them to move freely without resistance from gravity. Movement in this environment is not comparable to walking; nevertheless, the results signal significant progress towards the eventual goal of developing a therapy for a wide range of individuals with spinal cord injury. “These encouraging results provide continued evidence that spinal cord injury may no longer mean a life-long sentence of paralysis and support the need for more research,” said Roderic Pettigrew, Ph.D., M.D., director of the National Institute of Biomedical Imaging and Bioengineering at NIH. “The potential to offer a life-changing therapy to patients without requiring surgery would be a major advance; it could greatly expand the number of individuals who might benefit from spinal stimulation. It’s a wonderful example of the power that comes from combining advances in basic biological research with technological innovation.”
Link ID: 21242 - Posted: 08.01.2015
By Smitha Mundasad Health reporter A type of diabetes drug may offer a glimmer of hope in the fight against Parkinson's disease, research in the journal Plos Medicine suggests. Scientists found people taking glitazone pills were less likely to develop Parkinson's than patients on other diabetes drugs. But they caution the drugs can have serious side-effects and should not be given to healthy people. Instead, they suggest the findings should prompt further research. 'Unintended benefits' There are an estimated 127,000 people in the UK with Parkinson's disease, which can lead to tremor, slow movement and stiff muscles. And charities say with no drugs yet proven to treat the condition, much more work is needed in this area. The latest study focuses solely on people with diabetes who did not have Parkinson's disease at the beginning of the project. Researchers scoured UK electronic health records to compare 44,597 people prescribed glitazone pills with 120,373 people using other anti-diabetic treatment. They matched participants to ensure their age and stage of diabetes treatment were similar. Scientists found fewer people developed Parkinson's in the glitazone group - but the drug did not have a long-lasting benefit. Any potential protection disappeared once patients switched to another type of pill. Dr Ian Douglas, lead researcher at the London School of Hygiene and Tropical Medicine, said: "We often hear about negative side-effects associated with medications, but sometimes there can also be unintended beneficial effects. "Our findings provide unique evidence that we hope will drive further investigation into potential drug treatments for Parkinson's disease." © 2015 BBC
Link ID: 21199 - Posted: 07.22.2015
Results from tests of the drug, announced this week, show that it breaks up plaques in mice affected with Alzheimer’s disease or Parkinson’s disease, and improves the memories and cognitive abilities of the animals. Other promising results in rats and monkeys mean that the drug developers, NeuroPhage Pharmaceuticals, are poised to apply for permission to start testing it in people, with trials starting perhaps as early as next year. The drug is the first that seems to target and destroy the multiple types of plaque implicated in human brain disease. Plaques are clumps of misfolded proteins that gradually accumulate into sticky, brain-clogging gunk that kills neurons and robs people of their memories and other mental faculties. Different kinds of misfolded proteins are implicated in different brain diseases, and some can be seen within the same condition (see “Proteins gone rogue”, below). One thing they share, however, is a structural kink known as a canonical amyloid fold, and it is this on which the new drug acts (Journal of Molecular Biology, DOI: 10.1016/j.jmb.2014.04.015). Animal tests show that the drug reduces levels of amyloid beta plaques and tau protein deposits implicated in Alzheimer’s disease, and the alpha-synuclein protein deposits thought to play a role in Parkinson’s disease. Tests on lab-made samples show that the drug also targets misfolded transthyretin, clumps of which can clog up the heart and kidney, and prion aggregates, the cause of CJD, another neurodegenerative condition. Because correctly folded proteins do not have the distinct “kink”, the drug has no effect on them. © Copyright Reed Business Information Ltd.
Carl Zimmer A single neuron can’t do much on its own, but link billions of them together into a network and you’ve got a brain. But why stop there? In recent years, scientists have wondered what brains could do if they were linked together into even bigger networks. Miguel A. Nicolelis, director of the Center for Neuroengineering at Duke University, and his colleagues have now made the idea a bit more tangible by linking together animal brains with electrodes. In a pair of studies published on Thursday in the journal Scientific Reports, the researchers report that rats and monkeys can coordinate their brains to carry out such tasks as moving a simulated arm or recognizing simple patterns. In many of the trials, the networked animals performed better than individuals. “At least some times, more brains are better than one,” said Karen S. Rommelfanger, director of the Neuroethics Program at the Center for Ethics at Emory University, who was not involved in the study. Brain-networking research might someday allow people to join together in useful ways, Dr. Rommelfanger noted. Police officers might be able to make collective decisions on search-and-rescue missions. Surgeons might collectively operate on a single patient. But she also warned that brain networks could create a host of exotic ethical quandaries involving privacy and legal responsibility. If a brain network were to commit a crime, for example, who exactly would be guilty? © 2015 The New York Times Company
Link ID: 21160 - Posted: 07.11.2015
Skinny jeans can seriously damage muscles and nerves, doctors have said. A 35-year-old woman had to be cut out of a pair after her calves ballooned in size, the medics said in the Journal of Neurology, Neurosurgery and Psychiatry. She had spent hours squatting to empty cupboards for a house move in Australia. By evening, her feet were numb and she found it hard to walk. Doctors believe the woman developed a condition called compartment syndrome, made worse by her skinny jeans. Compartment syndrome is a painful and potentially serious condition caused by bleeding or swelling within an enclosed bundle of muscles - in this case, the calves. The condition caused the woman to trip and fall and, unable to get up, she then spent several hours lying on the ground. On examination at the Royal Adelaide Hospital, her lower legs were severely swollen. Although her feet were warm and had enough blood supplying them, her muscles were weak and she had lost some feeling. As the pressure had built in her lower legs, her muscles and nerves became damaged. She was put on an intravenous drip and after four days was able to walk unaided. Other medics have reported a number of cases where patients have developed tingly, numb thighs from wearing the figure-hugging low-cut denim trousers - although the chance of it happening is still slim for most people. Priya Dasoju, professional adviser at the Chartered Society of Physiotherapy, said: "As with many of these warnings, the very unfortunate case highlighted is an extreme one. © 2015 BBC
Keyword: Movement Disorders
Link ID: 21084 - Posted: 06.23.2015
by Laura Sanders The motor homunculus is a funny-looking fellow with a hulking thumb, delicate toes and a tongue that wags below his head. His body parts and proportions stem from decades-old experiments that mapped brain areas to the body parts they control. Now, a new study suggests that the motor homunculus’ neck was in the wrong place. Hyder Jinnah of Emory University in Atlanta and colleagues used fMRI to scan the brains of volunteers as they activated their head-turning neck muscles. (Pads held participants’ heads still, so the muscles fired but heads didn’t move.) This head turn was accompanied by activity in part of the brain that controls movement. The exact spot seems to be between the brain areas that control the shoulder and the trunk — not between the areas responsible for moving the thumb and the top of the head as earlier motor homunculi had suggested, the team reports in the June 17 Journal of Neuroscience. © Society for Science & the Public 2000 - 2015.
Keyword: Pain & Touch
Link ID: 21060 - Posted: 06.17.2015
by Penny Sarchet Simon Sponberg of Georgia Institute of Technology in Atlanta and his team have figured out the secret to the moths' night vision by testing them with robotic artificial flowers (see above). By varying the speed of a fake flower's horizontal motion and changing brightness levels, the team tested moths' abilities under different conditions. It has been theorised that the moth brain slows down, allowing their visual system to collect light for longer, a bit like lengthening a camera's exposure. But the strategy might also introduce blur, making it hard to detect fast movement. If the moths were using this brain-slowing tactic, they would be expected to react to fast flower movements more slowly in darker conditions. The team found that there was indeed a lag. It helped them see motion in the dark while still allowing them to keep up with flowers swaying at normal speeds. The size of the lags matched the expected behaviour of a slowed nervous system, providing evidence that moths could be slowing down the action of neurons in their visual system. Previously, placing hawkmoths in a virtual obstacle courseMovie Camera revealed that they vary their navigation strategies depending on visibility conditions. Journal reference: Science, DOI: 10.1126/science.aaa3042 © Copyright Reed Business Information Ltd
Link ID: 21041 - Posted: 06.13.2015
By Sandra G. Boodman The test had become something of an annual ritual. Every year beginning when he turned 45, Thomas Clark Semmes, an IT consultant for the federal government, would visit his internist for a physical. In a standard test of the sensory system that is often part of a physical, the Baltimore doctor would prick the soles of Semmes’s feet with a pin. “He’d look at me and say, ‘Tell me when you feel it,’ and I’d say ‘I will when I can,’ ” Semmes, now 56, recalled of the pinprick test. Because he never felt anything, he said nothing. “It never really concerned me very much,” he recalled. His doctor would then dutifully jot something in his chart, never exploring it further. But in 2013, nearly a decade after that first test, a quick evaluation by a podiatrist revealed the reason for his unfeeling feet and provided an explanation for an anatomical oddity in one of Semmes’s close relatives. In retrospect, Semmes wishes he had asked his internist about the lack of sensation, but he assumed it wasn’t important — otherwise, the doctor would have said something. And as Semmes would later learn, not knowing what was wrong had cost him valuable time. “I definitely wish I’d been diagnosed sooner,” he said. “There are things that could have been done to lessen the impact.” Before 2013, Semmes never had much reason to think about his feet. He knew he had hammertoes — toes that bend downward at the middle joint as a result of heredity or trauma — as well as extremely high arches, but neither condition was painful or limiting. At least, he thought, he did not have bird legs like his father, whose limbs were so storklike that they were a running family joke. “I had big, muscular legs,” Semmes said.
Fergus Walsh Medical correspondent Scientists in Austria have created an artificial leg which allows the amputee to feel lifelike sensations from their foot. The recipient, Wolfang Rangger, who lost his right leg in 2007, said: "It feels like I have a foot again. It's like a second lease of life." Prof Hubert Egger of the University of Linz, said sensors fitted to the sole of the artificial foot, stimulated nerves at the base of the stump. He added it was the first time that a leg amputee had been fitted with a sensory-enhanced prosthesis. How it works Surgeons first rewired nerve endings in the patient's stump to place them close to the skin surface. Six sensors were fitted to the base of the foot, to measure the pressure of heel, toe and foot movement. These signals were relayed to a micro-controller which relayed them to stimulators inside the shaft where it touched the base of the stump. These vibrated, stimulating the nerve endings under the skin, which relayed the signals to the brain. Prof Egger said: "The sensors tell the brain there is a foot and the wearer has the impression that it rolls off the ground when he walks." Wolfgang Ranger, a former teacher, who lost his leg after a blood clot caused by a stroke, has been testing the device for six months, both in the lab and at home. He says it has given him a new lease of life He said: "I no longer slip on ice and I can tell whether I walk on gravel, concrete, grass or sand. I can even feel small stones." © 2015 BBC.
by Helen Thomson Imagine a world where you think of something and it happens. For instance, what if the moment you realise you want a cup of tea, the kettle starts boiling? That reality is on the cards, now that a brain implant has been developed that can decode a person's intentions. It has already allowed a man paralysed from the neck down to control a robotic arm with unprecedented fluidity. But the implications go far beyond prosthetics. By placing an implant in the area of the brain responsible for intentions, scientists are investigating whether brain activity can give away future decisions – before a person is even aware of making them. Such a result may even alter our understanding of free will. Fluid movement "These are exciting times," says Pedro Lopes, who works at the human-computer interaction lab at Hasso Plattner Institute in Potsdam, Germany. "These developments give us a glimpse of an exciting future where devices will understand our intentions as a means of adapting to our plans." The implant was designed for Erik Sorto, who was left unable to move his limbs after a spinal cord injury 12 years ago. The idea was to give him the ability to move a stand-alone robotic arm by recording the activity in his posterior parietal cortex – a part of the brain used in planning movements. "We thought this would allow us to decode brain activity associated with the overall goal of a movement – for example, 'I want to pick up that cup'," Richard Andersen at the California Institute of Technology in Pasadena told delegates at the NeuroGaming Conference in San Francisco earlier this month. © Copyright Reed Business Information Ltd
by Andy Coghlan A man in his mid-50s with Parkinson's disease had fetal brain cells injected into his brain last week. He is the first person in nearly 20 years to be treated this way – and could recover full control of his movements in roughly five years. "It seemed to go fine," says Roger Barker of the University of Cambridge, who is leading the international team that is reviving the procedure. The treatment was pioneered 28 years ago in Sweden, but two trials in the US reported no significant benefit within the first two years following the injections, and the procedure was abandoned in favour of deep brain stimulation treatments. What these trials overlooked is that it takes several years for fetal cells to "bed in" and connect properly to the recipient's brain. Many Swedish and North American recipients improved dramatically, around three years or more after the implants – long after the trials had finished. "In the best cases, patients who had the treatment pretty much went back to normal," says Barker. After the fetal cells were wired up properly in their brains, they started producing the brain signalling chemical dopamine – low levels of this cause the classic Parkinson's symptom of uncontrolled movements. In fact, the cells produced so much dopamine that many patients could stop taking their Parkinson's drugs. "The prospect of not having to take medications for Parkinson's is fantastic," says James Beck of the Parkinson's Disease Foundation in the US. © Copyright Reed Business Information Ltd
by Helen Thomson A brain implant that can decode what someone wants to do has allowed a man paralysed from the neck down to control a robotic arm with unprecedented fluidity – and enjoy a beer at his own pace. Erik Sorto was left unable to move any of his limbs after an accident severed his spinal cord 12 years ago. People with similar injuries have previously controlled prosthetic limbs using implants placed in their motor cortex – an area of the brain responsible for the mechanics of movement. This is far from ideal because it results in delayed, jerky motions as the person thinks about all the individual aspects of the movement. When reaching for a drink, for example, they would have to think about moving their arm forward, then left, then opening their hand, then closing their hand around the cup and so on. Richard Andersen at the California Institute of Technology in Pasadena and his colleagues hoped they could achieve a more fluid movement by placing an implant in the posterior parietal cortex – a part of the brain involved in planning motor movements. "We thought this would allow us to decode brain activity associated with the overall goal of a movement – for example, 'I want to pick up that cup', rather than the individual components," said Anderson at the NeuroGaming Conference in San Francisco, California, where he presented the work this month. © Copyright Reed Business Information Ltd.
Link ID: 20972 - Posted: 05.23.2015
Scientists at Mayo Clinic, Jacksonville, Florida created a novel mouse that exhibits the symptoms and neurodegeneration associated with the most common genetic forms of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS, Lou Gehrig’s disease), both of which are caused by a mutation in the a gene called C9ORF72. The study was partially funded by the National Institutes of Health and published in the journal Science. More than 30,000 Americans live with ALS, which destroys nerves that control essential movements, including speaking, walking, breathing and swallowing. After Alzheimer’s disease, FTD is the most common form of early onset dementia. It is characterized by changes in personality, behavior and language due to loss of neurons in the brain’s frontal and temporal lobes. Patients with mutations in the chromosome 9 open reading frame 72 (C9ORF72) gene have all or some symptoms associated with both disorders. “Our mouse model exhibits the pathologies and symptoms of ALS and FTD seen in patients with theC9ORF72 mutation,” said the study’s lead author, Leonard Petrucelli, Ph.D., chair and Ralph and Ruth Abrams Professor of the Department of Neuroscience at Mayo Clinic, and a senior author of the study. “These mice could greatly improve our understanding of ALS and FTD and hasten the development of effective treatments.” To create the model, Ms. Jeannie Chew, a Mayo Graduate School student and member of Dr. Petrucelli’s team, injected the brains of newborn mice with a disease-causing version of the C9ORF72 gene. As the mice aged, they became hyperactive, anxious, and antisocial, in addition to having problems with movement that mirrored patient symptoms.