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TROY, NY - The cause of Lou Gehrig's Disease (ALS or amyotrophic lateral sclerosis) has remained elusive since it brought down one of baseball's greatest players 60 years ago. According to Wilfredo "Freddie" Colón, ALS starts "when good proteins go bad." Understanding just why they go bad is a necessary first step toward developing medicines that will help ALS patients live with a manageable disease instead of a death sentence. The Rensselaer biochemist's vital research has recently earned a $1 million, four-year grant from the National Institutes of Health. Copyright © 1996–2001 Rensselaer Polytechnic Institute.
Results Hold Possibilities For Treating Parkinson’s and Lou Gehrig’s Disease HOUSTON, Jan. 18, 2002 – New research from University of Houston scientists may lead to techniques for jump-starting the faulty "wiring" in damaged nerve cells, and suggests possible avenues for treating spinal cord injuries, Parkinson’s disease and amyotrophic lateral sclerosis, or ALS, also known as Lou Gehrig’s disease. University of Houston scientists studying how spinal nerve cells in chicken embryos develop and function have found that chemicals called growth factors play a key role in regulating how embryonic nerve cells acquire the ability to start processing information. "In some cases, when nerves are damaged or succumb to neurodegenerative diseases such as ALS and Parkinson’s, they don’t die, but they quit working and may actually revert to an immature embryonic-like state," says Stuart Dryer, a neuroscientist in the department of biology and biochemistry at UH. Embryonic nerve cells are able to fire electrical impulses shortly after the cells have divided for the last time – after they are "born." But these impulses are extremely generic, and not necessarily specialized for the kind of information the cell is going to eventually process, Dryer says.
By Katie Moisse Sprawling blooms of cyanobacteria have swathed the surfaces of lakes and oceans around the world for billions of years. But the serene, blue-green algae may be leaching a neurotoxin into the aquatic food chain, according to a study published May 3 in Proceedings of the National Academy of Sciences ( PNAS ). The report revived a nearly 50-year-old debate over the role, if any, of the toxin in the process of neurodegeneration. In the wake of World War II a deadly neurological disease plagued the small island of Guam. The natives called it lytico-bodig (from the Spanish paralytico, meaning weakness) and it had features of Lou Gehrig's (ALS), Parkinson's and Alzheimer's diseases. Endemic to the native population (called Chamorros), the syndrome was 100 times more prevalent on Guam than anywhere else. After ruling out a genetic cause, scientists began the hunt for an environmental trigger that made Chamorros, but not immigrants, susceptible. A staple of the local cuisine raised suspicion. Chamorros made tortillas using flour ground from the seeds of cycads—plants often confused for ferns or palms and distantly related to both. The seeds were meticulously washed to remove toxins, such as beta-methylamino-L-alanine (BMAA), produced by cyanobacteria that inhabit cycad roots. Scientists wondered if BMAA could be causing neurodegeneration, but the concentrations ingested by the Chamorros were not sufficient to harm neurons in animal models. Huge concentrations, however, were. © 2010 Scientific American,
By MARIA CHENG LONDON - Lithium doesn't help patients with ALS, or Lou Gehrig's disease, contrary to previous study results, new research says. Results from a small study published two years ago suggested the drug, often used for depression, could slow the fatal neurological disorder. Many ALS sufferers and their families rushed to try it, spearheading a patient-led effort to test lithium without doctors. In the first trial to scientifically assess whether lithium works for Lou Gehrig's disease, doctors found it had no effect — and stopped the study early because it seemed futile. The results were published online Tuesday in the medical journal, Lancet Neurology. American and Canadian doctors enrolled 84 patients with Lou Gehrig's disease into their study across both countries. About half the patients got lithium and riluzole, the standard drug used for the disease. The other half got riluzole plus placebo pills. After nearly six months, researchers didn't see any difference: 22 of the 40 patients in the lithium group had gotten worse versus 20 of 44 patients in the placebo group. Patients on lithium were more likely to have fallen and suffer back pain. The study was funded by the U.S. National Institute of Neurological Disorders and Stroke, the ALS Association and the ALS Society of Canada. Copyright 2010 The Associated Press.
By MICHELE MORGAN BOLTON MIDDLEBOROUGH, Mass., — The big news in this struggling southeastern Massachusetts community is a proposed $1 billion casino complex that many hope will bring financial salvation. But for a small group of residents, the hope for economic revival is overshadowed by health concerns. They are awaiting a report later this year that could reveal whether the dozens of cases of Lou Gehrig’s disease centered around a downtown industrial area were caused by pollution. The cases, which both state and federal officials call a disease cluster, are located within a mile of Everett Square — a densely settled neighborhood adjacent to the town’s onetime factory row. It is now home to two Superfund sites. The study, which was financed by the federal Agency for Toxic Substances and Disease Registry and conducted by state health scientists, will be followed by the creation of a statewide registry to track cases of the disease, formally known as amyotrophic lateral sclerosis, the cause of which is not fully understood. State Senator Marian Walsh, a Democrat from West Roxbury, said it was understandable that most residents were more interested in the prospect of obtaining a casino, which would be built by the Mashpee Wampanoag Indians and is expected to create as many as 10,000 jobs. “It’s human nature that we move toward pleasure and away from pain,” Ms. Walsh said. “But here, if we can understand the genesis, the registry will bring in money, information and resources that will help get to a cure.” Copyright 2007 The New York Times Company
Heidi Ledford Knocking out a single gene nearly doubles the lifespan of mice with the animal model of Lou Gehrig's disease, suggesting that the gene may one day become a target for therapies in humans. Lou Gehrig's disease, otherwise known as amyotrophic lateral sclerosis (ALS), is a neurodegenerative disease that gradually erodes motor control. Death usually follows within three to five years of diagnosis. There is no cure, and the only drug available that slows progression of the disease, riluzole, prolongs survival only by a few months. Mice develop ALS-like symptoms when they have a mutation in a gene called SOD1 — a mutation that causes about 1-2% of human ALS cases. Research using these animal models has suggested that chemically reactive forms of oxygen that can damage cells also contribute to the disease. Several proteins present in the bodies of mice and people are known to generate reactive oxygen species as part of their normal function in cell signalling and inflammation. So John Engelhardt and his colleagues at the University of Iowa in Iowa City decided to look closely at two of these — Nox1 and Nox2 — to see whether turning down the amount of such proteins could slow the progression of ALS symptoms. It did — dramatically. The team found that ALS mice lacking the gene that creates Nox2 produced fewer reactive oxygen species and lived on average for 229 days — 97 days longer than those who had normal levels of Nox21. ©2007 Nature Publishing Group
Michael Hopkin British neuroscientists are planning to investigate whether playing soccer contributes to the development of motor neurone disease. The move comes after three amateur footballers playing in the same league developed the disease, which normally affects less than one person in every 50,000 each year. Experts are now aiming to launch a full epidemiological study of professional footballers and motor neurone disease (MND) patients, to see whether the sport really does raise the incidence of the disease among those who play it at a high level. Details of the patients, all of whom were committed footballers, are published in the journal Amyotrophic Lateral Sclerosis1 — a publication named after the most common form of MND. The patients range in age between 56 and 61 years old, and were all diagnosed with the disease within a decade of each other. "What is unusual about this group is that they are all friends who developed MND at the same time," says Ammar Al-Chalabi of King's College London, one of the experts who described the cases. "A cluster like this could occur by chance, but the odds are quite long." The three have several potential risk factors in common, including having been electrocuted by mains electricity at some point during their lives. But the authors note that the three were very keen at football, playing more than twice a week — almost as much as professional players. ©2007 Nature Publishing Group
In 1979 Jack O’Neil, 69, from New York City, was diagnosed with Lou Gehrig's disease, also known as ALS, or amytrophic lateral sclerosis. "The prognosis indicated that I had between two and five years to live," says O'Neil. Fortunately the disease was unusually slow to affect him, allowing him to keep working for more than 15 years. But finally in 1985 he was forced to stop working as he lost control of his muscles. "I had no energy. I couldn't fulfill my work obligations and I couldn't travel. I was just exhausted all the time," O'Neil remembers. "I could walk a little bit, but gradually I didn't have strength to do anything—to move my hands or my arms or anything like that." ALS, a rapidly progressive, invariably fatal neurological disease, is one of the most common neuromuscular diseases worldwide. The specialized nerve cells of the spine that control muscle movements in the body selectively die, leading to progressive paralysis. It strikes people mostly between 40 and 60 years of age, but sometimes even younger, and typically more men than women. As many as 20,000 Americans have ALS and around 5,000 Americans are diagnosed each year. © ScienCentral, 2000- 2004.
— The life or death of motor neurons in patients afflicted with amyotrophic lateral sclerosis (ALS) may rest with a somewhat overlooked group of support cells that helps guide, nourish and remove toxins from neurons. Howard Hughes Medical Institute researchers and their colleagues have discovered that non-neuronal cells, called astrocytes and glia, can protect neurons containing ALS-causing mutations from degeneration. Their studies also show that if non-neuronal cells harbor ALS mutations, then damage can occur in neighboring motor neurons that are otherwise healthy. According to the researchers, their findings suggest that it may be possible to insert healthy astrocytes into ALS patients to reduce or prevent motor-neuron degeneration. Motor neurons control muscle action, and they are progressively weakened in ALS, leading to paralysis and death. ©2003 Howard Hughes Medical Institute
La Jolla, Calif.—Manufacturing motor nerve cells may someday be possible to help restore function in victims of spinal cord injury or such diseases of motion as Parkinson’s and Lou Gehrig’s disease or post-polio syndrome, a Salk Institute research study has found. Salk Associate Professor Sam Pfaff and postdoctoral fellow Soo-Kyung Lee reported in a paper in the June 5 issue of Neuron that they constructed a detailed model of how stem cells are prodded, regulated and otherwise encouraged to become not only nerve cells, but specifically motor neurons that the body relies on to move muscles and limbs throughout the body. The study provides the first blueprint for the cellular factory that produces motor neurons from embryonic stem cells. It could eventually result in new treatments for spinal cord injury, and other diseases that affect motor nerve cells.
Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 7: Life-Span Development of the Brain and Behavior
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 13: Memory, Learning, and Development
Link ID: 3890 - Posted: 06.24.2010
Eating bat meat may be to blame for mystery illness ASSOCIATED PRESS WASHINGTON, — Scientists have long sought to understand a horrific brain disease that once devastated the native people of Guam — Lou Gehrig’s, Alzheimer’s and Parkinson’s symptoms rolled into one. Now two researchers have uncovered clues that suggest a Chamorro dietary tradition — eating a type of bat that feeds on neurotoxic plants — might be behind the mystery illness. IT’S CIRCUMSTANTIAL evidence so far. But if the new theory is proved right, it could be more than another dismal discovery that diet can wreck the human brain. Understanding the Guam disease may help uncover novel ways to treat regular Lou Gehrig’s, Alzheimer’s or Parkinson’s diseases. The theory, published Monday in the journal Neurology, turns on the principle that changing economies can impact disease. The brain disease peaked after World War II brought guns and cash to Guam, spurring commercial hunting until the bats neared extinction — and then the human disease in turn rapidly waned, said ethnobotanist Paul Alan Cox, who studies how indigenous people relate to their environment. • MSNBC Terms, Conditions and Privacy © 2002
Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 4: The Chemistry of Behavior: Neurotransmitters and Neuropharmacology
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 4: The Chemistry of Behavior: Neurotransmitters and Neuropharmacology
Link ID: 1754 - Posted: 06.24.2010
By GEOFF DAVIES A Dalhousie University professor and an international team of researchers have discovered what makes us kick. Dr. Rob Brownstone, along with colleagues in New York and Scotland, discovered a group of nerve cells that are critical to regulating how much force muscles use when performing movements. "We knew that they had to be there," Dr. Brownstone said Friday, roughly a week after Neuron, the world’s leading neuroscience journal, published the findings. "But we couldn’t pinpoint them and we couldn’t say exactly what their role was in a behaving animal." The researchers located a group of cells that regulate how much force is used by motor neurons, nerve cells in the spinal cord that make muscles contract. The team used genetic techniques to locate and deactivate these new-found "modulatory" cells in mice. "When we did that, that’s when we found that the animals couldn’t contract their muscles in their legs as much as they needed to swim properly," Dr. Brownstone said. "This is a fundamental discovery about how the spinal cord works to produce movement." Further on down the line, this discovery could lead to breakthroughs in the treatment of Lou Gehrig’s disease, spinal cord injuries and other conditions, Dr. Brownstone said. © 2009 The Halifax Herald Limited
By Terry J. Allen Researchers investigating a deadly disease cluster near a New Hampshire lake are tracking clues that stretch from a delicacy eaten on Guam to a 3.5 billion-year-old type of bacteria and the green scum that coats many New England waters. The scum - blooms of cyanobacteria often misnamed blue-green algae - produces a toxin that doctors at Dartmouth-Hitchcock Medical Center in Lebanon, N.H., suspect might have triggered cases of amyotrophic lateral sclerosis along the north shore of nearby Mascoma Lake. Using patient records and mapping software, the Dartmouth-Hitchcock team looked for ALS clusters in Maine, New Hampshire, and Vermont. Their preliminary data suggest that the disease, also known as Lou Gehrig’s disease, is about 2.5 times more prevalent among people who live within a half-mile of water bodies with past or current cyanobacteria colonies. The incidence of ALS was highest near Mascoma Lake, where nine patients have been diagnosed since 1990, all but one since 2000 - a rate at least 10 times the US average of two in 100,000 people diagnosed annually. The neurodegenerative disease eventually immobilizes patients and, inevitably, destroys their ability to swallow and breathe. In one survey, doctors said ALS is the diagnosis they most dread giving. © 2009 NY Times Co.
The fatal disorder ALS wreaks its havoc by harming nerve cells that control muscles. As a consequence of the damage, patients experience progressive muscle weakness that can hinder movement and speech, even swallowing and breathing. Once, little could be done to help these patients who often spend their last days in an isolating paralysis. But now following years of research, scientists are finding ways to shield cells from the wrath of ALS and help delay the disorder’s progression. Recent developments include the potential use of growth factors, special substances that appear to offer cell protection. No crime was committed. Yet an estimated 5,000 Americans afflicted annually with ALS will suffer a particularly torturous sentence. People with this disorder, also known as amyotrophic lateral sclerosis or Lou Gehrig’s disease, often end up imprisoned in their bodies. Many spend their last days completely unable to move while their minds remain sharp and alert. ALS wreaks its havoc by harming nerve cells that control muscles. As a consequence of the damage, people with ALS experience progressive muscle weakness that can hinder movement and speech, creating an isolating paralysis. Swallowing and breathing also may be harmed and many die within three to five years after they experience symptoms, usually in mid-life. Once, doctors could do little to help these patients. But now following years of study, scientists are finding ways to protect cells and help delay the progression of ALS. Copyright © 2005 Society for Neuroscience
the virtues of proteins that can save dying neurons Diseases characterized by neurodegeneration affect individuals over 50 years of age and they attack one particular class of neurons in the brain or spinal cord. The research interest of Professor Ann Kato and her team is principally focalized on ALS in which there is a progressive paralysis caused by the destruction of motor neurons which exist in both the brain and spinal cord. These cells are responsible for the movements of the upper and lower limbs and their elimination causes muscular atrophy and finally death. During the last 3 years, the objectives of the experiments of Professor Kato consist in trying to understand why these neurons die and to study the intracellular and molecular pathways which can prevent their degeneration. It was during the course of their experiments to attempt to prolong the survival of the cells that the group of Professor Kato found that a newly discovered family of proteins called Inhibitors of Apoptosis Proteins (IAP’s) were capable of preventing the death of motor neurons. Nature Cell Biology, February 2002, vol. 4, and pages175-179 © AlphaGalileo 2001
Related chapters from BP7e: Chapter 11: Motor Control and Plasticity; Chapter 7: Life-Span Development of the Brain and Behavior
Related chapters from MM:Chapter 5: The Sensorimotor System; Chapter 13: Memory, Learning, and Development
Link ID: 1426 - Posted: 06.24.2010
Perhaps one of the most astonishing features of the human nervous system is the fact that muscles in one part of the body, for example the feet, can be controlled by neurons whose cell bodies are located extremely far away in the spinal cord. These cell bodies therefore must extend processes incredibly long distances. As most of the proteins in a neuron are made in the cell body, the transport of proteins and other molecules through these very long processes called axons is critically important for proper motor control. In certain neurodegenerative diseases in which motor control is impaired, e.g., amyotrophic lateral sclerosis, traffic along the axons slows down and certain molecules accumulate. Could this cellular “traffic jam” be responsible for the degenerative phenotype and associated muscular atrophy? Copyright © 1995-2002 UniSci. All rights reserved.
By MARCUS WOHLSEN CLAREMONT, Calif. -- Until last year, Alan Felzer was an energetic engineering professor who took the stairs to his classes two steps at a time. Now the 64-year-old grandfather sits strapped to a wheelchair, able to move little but his left hand, his voice a near-whisper. Felzer suffers from ALS, also known as Lou Gehrig's disease. The fatal neurological disorder steals the body's ability to move, speak and ultimately to breathe. But rather than succumb to despair along with his illness, Felzer turned to the Web to become his own medical researcher _ and his own guinea pig. Dozens of ALS patients are testing treatments on their own without waiting on the slow pace of medical research. They are part of an emerging group of patients willing to share intimate health details on the Web in hopes of making their own medical discoveries. Some doctors caution that such patient-led research lacks rigor and may lead to unreliable results, false hopes and harm to patients. "The Internet is a wonderful tool, but you know, it's buyer beware," said Dr. Edward Langston, immediate past chairman of the American Medical Association's board. In Felzer's case, the experiment's results illustrate the obstacles that stand between patients and self-discovered breakthroughs. The drug he tried did no good. But he and his family felt they had little time and little to lose in trying. © Copyright 1996-2008 The Washington Post Company
By Claire Panosian Dunavan When my friend Nikki Tal died in 2004, the world lost a strong, brave soul -- despite a thuggish disease that had by then utterly ravaged her body. From her earliest years, Nikki had been a reader. She also loved the ocean. Her favorite book was "Life of Pi," about a shipwrecked boy trapped on a raft with a hungry predator. The parallels with her own life weren't hard to see. Nikki's predator was amyotrophic lateral sclerosis, better known as ALS, or Lou Gehrig's disease. As any doctor will tell you, for patients with neurologic blights, ALS is about as bad as it gets. In less than a year, it can extinguish an entire set of motor neurons, rendering its victims limp and powerless. It was October 1996 when Nikki first learned something was wrong. While attending a baby shower, the 5-foot-8 lawyer realized she couldn't lift a 10-pound newborn. Later that month, she destroyed her car's ignition after repeatedly mis-inserting her key; then she begged off timing duty at her daughter's swim meets because she could no longer depress a stopwatch. A few months later came the inability to walk or care for herself; the big, padded wheelchair and the handicap van; and, eventually, the feeding tube and the portable ventilator. Finally, ALS robbed Nikki of the ability to hold her head erect and of producing even remotely intelligible speech. © 2008 The Washington Post Company
By Devin Powell Even with the entire human genome in hand, scientists can still have trouble rooting out the genes behind a disease. Consider amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. One ALS-related gene mutation was found in 1993, but it affects only 1% of all ALS patients. Now, after a long dry spell, researchers have finally hit on a second. It's just as rare as the first, but it seems to be more closely related to aspects of ALS pathology found in all patients. ALS typically appears in middle age, slowly destroying the motor neurons used to control muscles and usually killing a patient within 5 years of diagnosis. Five percent of all adult-onset ALS cases are known to have a strong genetic component, affecting multiple family members. But the rest seem to appear spontaneously in people with no prior family history. Scientists know little about what causes these sporadic cases. And the one known ALS gene in inherited ALS, called SOD1, doesn't seem to lead to neuronal death--the primary characteristic of ALS--so the link between this gene and the disease is still unclear. Christopher Shaw, a neurologist at King's College London, started hunting for other ALS genes by recruiting patients who have the inherited form of the disease but not the SOD1 mutation. While screening 154 people with familial ALS, Shaw and his colleagues found four individuals in one family who shared the same mutation: a single changed base on chromosome 1. The base was located in the TDPB gene, which encodes a protein called TDP-43 whose function isn't clear. In 2006, scientists reported that in both inherited and sporadic ALS, this protein disappears from the nucleus and clumps up in the cytoplasm of brain and motor neurons. © 2008 American Association for the Advancement of Science.
A rigorous study in Italy has confirmed claims that professional soccer players have a higher than normal risk of developing a type of motor neuron disease, also known as amyotrophic lateral sclerosis. The reason remains a mystery. ALS involves the death of motor neurons, the nerve cells responsible for voluntary movement, and eventually leads to paralysis and death. Adriano Chiò's team at the University of Turin surveyed the medical records of 7000 professional footballers who played in Italy's first or second division between 1970 and 2001. Based on the normal incidence of the disease and the players' ages, the researchers calculated that there should have been 0.8 cases of ALS in this group. Instead, there were five. The study was prompted by what the Italian press dubbed "the motor neuron mystery" - the discovery a few years ago of 33 cases of ALS during an investigation of illicit drug use among 24,000 pro and semi-pro players in Italy. Dubious about the methodology of that initial investigation, Chiò's group applied stricter diagnostic criteria to their data, such as only including players born in Italy. "I think the researchers have been conservative," says Ammar Al-Chalabi of the Institute of Psychiatry in London, who has written a commentary on the study in Brain. © Copyright Reed Business Information Ltd.