ScienceDaily: Amyotrophic Lateral Sclerosis News

Neon exposes hidden ALS cells

Tue, 30 Apr 2013 19:43:43 EDT

A small group of neurons in the cortex play a big role in ALS (amyotrophic lateral sclerosis), a fatal disease. But the neurons have been difficult to study because they look so similar to others in the cortex. New research has isolated the brain's motor neurons that die in ALS and dressed them in a green fluorescent jacket. Now scientists can easily find them to study why they die and how to save them.

Melatonin delays ALS symptom onset and death in mice

Thu, 25 Apr 2013 09:16:16 EDT

Melatonin injections delayed symptom onset and reduced mortality in a mouse model of the neurodegenerative condition amyotrophic lateral sclerosis, or Lou Gehrig's disease, according to a new study.

ALS trial suggests novel therapy is safe

Tue, 23 Apr 2013 17:27:27 EDT

An investigational treatment for an inherited form of Lou Gehrig's disease has passed an early phase clinical trial for safety, researchers report.

A new approach for spinal muscular atrophy?

Tue, 09 Apr 2013 17:34:34 EDT

Spinal muscular atrophy is a debilitating neuromuscular disease that in its most severe form is the leading genetic cause of infant death. By experimenting with an ALS drug in two very different animal models, researchers have identified a new potential mechanism for developing an SMA treatment.

Phase 1 ALS trial is first to test antisense treatment of neurodegenerative disease: No serious adverse effects reported

Wed, 03 Apr 2013 14:14:14 EDT

The initial clinical trial of a novel approach to treating amyotrophic lateral sclerosis -- blocking production of a mutant protein that causes an inherited form of the progressive neurodegererative disease -- may be a first step towards a new era in the treatment of such disorders.

Mutations in VCP gene implicated in a number of neurodegenerative diseases

Wed, 13 Mar 2013 21:40:40 EDT

New research gives insight into how single mutations in the VCP gene cause a range of neurological conditions including a form of dementia called Inclusion Body Myopathy, Paget’s Disease of the Bone and Frontotemporal Dementia (IBMPFD), and the motor neuron disease Amyotrophic Lateral Sclerosis (ALS).

Mutated gene causes nerve cell death

Sun, 10 Mar 2013 16:38:38 EDT

The British astrophysicist Stephen Hawking is likely to be the world's most famous person living with amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. ALS is a progressive disease affecting motor neurons, nerve cells that control muscle function, and nearly always leads to death. Researchers have now identified a completely new mechanism in the onset of motor neuron diseases. Their findings could be the basis for future treatments for these presently incurable diseases.

Adding to the list of disease-causing proteins in brain disorders

Sun, 03 Mar 2013 15:49:49 EST

Researchers have found new candidate disease proteins for neurodegenerative disorders. They describe mutations in prion-like segments of two RNA-binding proteins are associated with a rare inherited degeneration disorder affecting muscle, brain, motor neurons and bone (called multisystem proteinopathy) and one case of the familial form of amyotrophic lateral sclerosis.

Identification of abnormal protein may help diagnose, treat ALS and frontotemporal dementia

Tue, 12 Feb 2013 12:18:18 EST

Amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease, and frontotemporal dementia (FTD) are devastating neurodegenerative diseases with no effective treatment. Researchers are beginning to recognize ALS and FTD as part of a spectrum disorder with overlapping symptoms. Now investigators have discovered an abnormal protein that first forms as a result of genetic abnormalities and later builds up in the brains of many patients with either disease.

Eating bright-colored fruits and vegetables may prevent or delay amyotrophic lateral sclerosis

Tue, 29 Jan 2013 08:05:05 EST

New research suggests that increased consumption of foods containing colorful carotenoids, particularly beta-carotene and lutein, may prevent or delay the onset of amyotrophic lateral sclerosis (ALS).

One form of neuron turned into another in brain

Sun, 20 Jan 2013 15:00:00 EST

A new finding by stem cell biologists turns one of the basics of neurobiology on its head -- demonstrating that it is possible to turn one type of already differentiated neuron into another within the brain.

New genetic mutation for amyotrophic lateral sclerosis identified

Tue, 15 Jan 2013 14:39:39 EST

Researchers have identified a new genetic mutation for amyotrophic lateral sclerosis (ALS), opening the door to future targeted therapies. Medical researchers found that mutations within the ARHGEF28 gene are present in ALS. When they looked across both familial and sporadic forms of the disease, they found that virtually all cases of ALS demonstrated abnormal inclusions of the protein that arises from this gene.

Stem cells may hold promise for Lou Gehrig’s disease (ALS)

Wed, 09 Jan 2013 18:56:56 EST

Apparent stem cell transplant success in mice may hold promise for people with amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease, according to results of a new study.

Removing protein 'garbage' in nerve cells may help control 2 neurodegenerative diseases

Thu, 20 Dec 2012 08:04:04 EST

A group of neuroscientists say they have new evidence that challenges scientific dogma involving two fatal neurodegenerative diseases -- amyotrophic lateral sclerosis, and frontotemporal dementia -- and, in the process, have uncovered a possible therapeutic target as a novel strategy to treat both disorders.

Discovery provides therapeutic target for amyotrophic lateral sclerosis

Wed, 19 Dec 2012 17:43:43 EST

Researchers have found that the ability of a protein made by a gene called FUS to bind to RNA is essential to the development of amyotrophic lateral sclerosis (ALS). This discovery identifies a possible therapeutic target for the fatal neurological disease.

Transplanted neural stem cells treat amyotrophic lateral sclerosis in mouse model

Wed, 19 Dec 2012 14:22:22 EST

Transplanting neural stem cells into an ALS mouse model slows disease progression and prolongs survival. The transplanted neural stem cells changed the host environment for the better and protected endangered nerve cells. These findings demonstrate the potential neural stem cells hold for treating ALS.

Toxic interaction in neurons that leads to dementia and ALS discovered

Mon, 10 Dec 2012 16:08:08 EST

Researchers have uncovered a toxic cellular process by which a protein that maintains the health of neurons becomes deficient and can lead to dementia. The findings shed new light on the link between culprits implicated in two devastating neurological diseases: frontotemporal dementia and amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease.

One gene predicts rapid ALS progression 80 percent of the time

Fri, 07 Dec 2012 16:14:14 EST

The debilitating symptoms of amyotrophic lateral sclerosis, or ALS, appear to be increased by a lack of inflammation-reducing T cells, and the expression of a gene that controls production of anti-inflammatory T cells (FoxP3) predict how quickly the disease will progress.

Protein test is first to predict rate of progression in Lou Gehrig's disease

Mon, 19 Nov 2012 13:22:22 EST

A novel test that measures proteins from nerve damage that are deposited in blood and spinal fluid reveals the rate of progression of amyotrophic lateral sclerosis (ALS) in patients.

New development in relief of spasms related to amyotrophic lateral sclerosis

Tue, 06 Nov 2012 10:20:20 EST

Scientists have recently discovered the origin of spasms - a disabling symptom of amyotrophic lateral sclerosis. The degeneration of serotonin-releasing neurons is responsible for these sensations. In the longer term, researchers imagine that molecules acting on serotonin receptors present in the brain could eliminate spasticity in patients.

Stay-at-home transcription factor prevents neurodegeneration

Mon, 29 Oct 2012 09:27:27 EDT

A new study shows how a transcription factor called STAT3 remains in the axon of nerve cells to help prevent neurodegeneration. The findings could pave the way for future drug therapies to slow nerve damage in patients with neurodegenerative diseases.

Yeast model offers clues to possible drug targets for Lou Gehrig's disease, study shows

Sun, 28 Oct 2012 14:23:23 EDT

Amyotrophic lateral sclerosis, also called Lou Gehrig's disease, is a devastatingly cruel neurodegenerative disorder that robs sufferers of the ability to move, speak and, finally, breathe. Now researchers have used baker's yeast -- a tiny, one-celled organism -- to identify a chink in the armor of the currently incurable disease that may eventually lead to new therapies for human patients.

Scientists block toxic protein that plays key role in Lou Gehrig's disease

Sun, 28 Oct 2012 14:17:17 EDT

Scientists have discovered how modifying a gene halts the toxic buildup of a protein found in nerve cells. These findings point to a potential new tactic for treating a variety of neurodegenerative conditions, including amyotrophic lateral sclerosis.

Voice prostheses can help patients regain their lost voice

Wed, 24 Oct 2012 09:30:30 EDT

Help is on the way for people who suffer from vocal cord dysfunction. Researchers are developing methods that will contribute to manufacturing voice prostheses with improved affective features. For example, for little girls who have lost their voices, the improved artificial voice devices can produce age-appropriate voices, instead of the usual voice of an adult male.

Swiss assisted suicide laws do not necessarily promote desire for death, study finds

Tue, 09 Oct 2012 09:25:25 EDT

A new study shows that while current Swiss law does not necessarily increase the desire for assisted suicide, patients wish to discuss the option with their physician.

Mice with Lou Gehrig's disease not quite what the doctors ordered

Fri, 05 Oct 2012 13:46:46 EDT

You’ve heard the tale before: Scientists can treat diseases like Alzheimer’s and Lou Gehrig’s in mice, but when those same drugs get to human trials, they fail. Can researchers come up with mice that better mimic the patient? In the case of Lou Gehrig’s, some of the latest mice have a problem: they die not because of their spinal nerve disease, but due to blockage of their gut.

Common RNA pathway found in ALS and dementia

Sun, 30 Sep 2012 14:21:21 EDT

Two proteins previously found to contribute to ALS, also known as Lou Gehrig's disease, have divergent roles. But a new study shows that a common pathway links them.

Shared pathway links Lou Gehrig's disease with spinal muscular atrophy

Thu, 27 Sep 2012 12:41:41 EDT

Scientists have long known the main proteins that lead to the development of amyotrophic lateral sclerosis and spinal muscular atrophy, respectively. Now research shows that these two motor neuron diseases likely share a pathway that leads to the development of disease.

Storm of 'awakened' transposons may cause brain-cell pathologies in ALS, other illnesses

Thu, 06 Sep 2012 12:32:32 EDT

A team of neuroscientists and informatics experts reports important progress in an effort to understand the relationship between transposons -- sequences of DNA that can jump around within the genome, potentially causing great damage -- and mechanisms involved in serious neurodegenerative disorders including ALS, FTLD (frontotemporal lobar degeneration) and Alzheimer's disease.

NFL players may be at higher risk of death from Alzheimer’s and ALS, research shows

Wed, 05 Sep 2012 16:33:33 EDT

New research shows that professional football players may be at a higher risk of death from diseases that damage the cells in the brain, such as Alzheimer’s disease and ALS (also known as Lou Gehrig’s disease), compared to the general U.S. population.

Scientists identify new gene that influences survival in amyotrophic lateral sclerosis

Sun, 26 Aug 2012 14:36:36 EDT

Scientists have discovered a gene that influences survival time in amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease).

Researchers discover blood biomarker for Lou Gehrig's disease, could lead to new treatments

Mon, 06 Aug 2012 13:05:05 EDT

Researchers have discovered that changes in monocytes (a type of white blood cell) are a biomarker for amyotrophic lateral sclerosis, or Lou Gehrig's disease. The findings open doors to possible new treatments.

Anacardic acid found to rescue certain ALS abnormalities in experimental drug screening assay using motor neurons from ALS patient-specific iPSCs

Thu, 02 Aug 2012 09:22:22 EDT

Researchers recapitulated ALS abnormalities in motor neurons derived from induced pluripotent stem cells obtained from familial ALS patients. They revealed some of the mechanism of disease onset, finding that, for example, Tar DNA-binding protein-43 (TDP-43) mRNA was upregulated in the ALS motor neurons, which means that TDP-43 autoregulation was disturbed. Using the motor neurons in a drug-screening assay, they discovered that the chemical compound anacardic acid rescued some abnormal ALS motor neuron phenotypes.

Discovery may lead to new treatment for ALS

Wed, 18 Jul 2012 17:24:24 EDT

Researchers have discovered that TDP-43, a protein strongly linked to ALS (amyotrophic lateral sclerosis) and other neurodegnerative diseases, appears to activate a variety of different molecular pathways when genetically manipulated.

Disruption of cytoskeleton pathways contribute to amyotrophic lateral sclerosis pathogenesis

Sun, 15 Jul 2012 14:14:14 EDT

A new genetic mutation that causes familial amyotrophic lateral sclerosis (ALS), a fatal neurological disorder also known as Lou Gehrig's Disease, has been identified. Mutations to the profilin gene, which is essential to the growth and development of nerve cell axons, points to defects in a neuron's cytoskeleton structure as a potential common feature among diverse ALS genes.

'Insulating' brain cells appear to play a critical role in brain cell survival and may contribute to neurodegenerative diseases such as ALS

Wed, 11 Jul 2012 13:09:09 EDT

Researchers say they have discovered that the central nervous system’s oligodendroglia cells, long believed to simply insulate nerves as they “fire” signals, are unexpectedly also vital to the survival of neurons. Damage to these insulators appears to contribute to brain injury in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease for the Yankee baseball great who died from the disease.

1960s-era anti-cancer drug points to treatments for Lou Gehrig's disease

Wed, 13 Jun 2012 13:32:32 EDT

A long-used anti-cancer drug could be a starting point to develop new treatments for the incurable nerve disease known as Lou Gehrig's disease or amyotrophic lateral sclerosis (ALS), scientists are reporting. Their research shows how the drug prevents clumping of an enzyme linked to ALS.

How immune system, inflammation may play role in Lou Gehrig's disease

Tue, 05 Jun 2012 12:17:17 EDT

In an early study, researchers found that the immune cells of patients with amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease, may play a role in damaging the neurons in the spinal cord. ALS is a disease of the nerve cells in the brain and spinal cord that control voluntary muscle movement.

Genetic mutation causing rare form of spinal muscular atrophy identified

Thu, 10 May 2012 11:35:35 EDT

Scientists have confirmed that mutations of a gene are responsible for some cases of a rare, inherited disease that causes progressive muscle degeneration and weakness: spinal muscular atrophy with lower extremity predominance, also known as SMA-LED.

ALS clinics start implanting breathing-assist device under new FDA approval

Wed, 02 May 2012 14:38:38 EDT

Three U.S. hospitals have begun implanting a device that stimulates the respiratory muscle in the chest and draws air into the lungs of patients suffering from amyotrophic lateral sclerosis (ALS, Lou Gehrig’s disease) under recently approved Food and Drug Administration guidelines.

Neurotoxins in shark fins: A human health concern

Thu, 23 Feb 2012 18:25:25 EST

Sharks are among the most threatened of marine species worldwide due to unsustainable overfishing. They are primarily killed for their fins to fuel the growing demand for shark fin soup, which is an Asia delicacy. A new study has discovered high concentrations of BMAA in shark fins, a neurotoxin linked to neurodegenerative diseases in humans including Alzheimer’s and Lou Gehrig Disease (ALS). The study suggests that consumption of shark fin soup and cartilage pills may pose a significant health risk for degenerative brain diseases.

Complex wiring of the nervous system may rely on a just a handful of genes and proteins

Sat, 11 Feb 2012 09:53:53 EST

Researchers have discovered a startling feature of early brain development that helps to explain how complex neuron wiring patterns are programmed using just a handful of critical genes. The findings may help scientists develop new therapies for neurological disorders, such as amyotrophic lateral sclerosis, and provide insight into certain cancers.

Worm reproduction offers new explanation of Lou Gehrig's disease

Thu, 19 Jan 2012 13:32:32 EST

In a discovery many neurobiologists once thought too farfetched to accept, a protein known to help sperm move toward eggs during conception of the worm C. elegans appears to play a central role in Lou Gehrig's disease, according to a new research.

New culprit found in Lou Gehrig's disease

Mon, 21 Nov 2011 19:40:40 EST

Following a breakthrough identifying a common converging point for all forms of Lou Gehrig's disease, a new finding from the same scientists shines more light on the broken recycling pathway of the brain and spinal cord cells, which leads to the paralysis of ALS. The new study reveals a second faulty gene in the same pathway, offering a clear target for drug therapy.

Potential new drug target in Lou Gehrig's disease

Mon, 14 Nov 2011 13:36:36 EST

Two proteins conspire to promote Lou Gehrig's disease, according to a new study.

Why do neurons die in Parkinson's disease? Study of hereditary Parkinson’s finds that mitochondria can’t be cleared out when damaged

Thu, 10 Nov 2011 14:21:21 EST

Current thinking about Parkinson's disease is that it's a disorder of mitochondria, the energy-producing organelles inside cells, causing neurons in the brain's substantia nigra to die or become impaired. A study now shows that genetic mutations causing a hereditary form of Parkinson's disease cause mitochondria to run amok inside the cell, leaving the cell without a brake to stop them.

Discovery helps explain progression of Lou Gehrig's disease, offers new therapy approach

Mon, 17 Oct 2011 15:56:56 EDT

Researchers have discovered a previously unknown type of neural cell that appears to be closely linked to the progression of amytrophic lateral sclerosis, or Lou Gehrig's disease, that they believe will provide an important new approach to therapies. There is now no treatment for this disease, which causes progressive death of motor neurons, serious debility, paralysis and ultimately death within a few years.

Gene for Lou Gehrig's disease and frontotemporal dementia identified

Wed, 21 Sep 2011 14:22:22 EDT

Frontotemporal dementia and amyotrophic lateral sclerosis, also known as Lou Gehrig's disease -- two fatal neurodegenerative disease with distinct symptoms -- are triggered by a common mutation in many cases, according to researchers who say they have identified the mutated gene.

New genetic mutation for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia

Wed, 21 Sep 2011 13:23:23 EDT

A team led by scientists has discovered a new genetic mutation for amyotrophic lateral sclerosis (ALS) and a related disease called frontotemporal dementia that appears to account for more than a third of all inherited cases of these diseases.

New genetic mutation is the most common cause of familial forms of frontotemporal dementia and ALS

Wed, 21 Sep 2011 13:23:23 EDT

Researchers have found a genetic abnormality they say is the most common cause of two different but related familial forms of neurodegenerative disease -- frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease.

Human genetics study identifies the most common cause of ALS and dementia

Wed, 21 Sep 2011 13:23:23 EDT

Scientists have made an exciting breakthrough in unraveling the genetic basis of two debilitating neurodegenerative disorders, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Two independent studies identify a new human genetic mutation as the most common cause of ALS and FTD identified to date.

MRI research demonstrates ALS attacks multiple parts of the brain

Fri, 16 Sep 2011 13:12:12 EDT

Medical researchers demonstrate that ALS -- known as Lou Gehrig's disease -- damages neurons in parts of the brain responsible for cognition and behavior. The scientists provide evidence that ALS affects more than just the motor cortex, the part of the brain responsible for motor function.

Two genes that cause familial ALS shown to work together

Thu, 01 Sep 2011 13:46:46 EDT

Although several genes have been linked to amyotrophic lateral sclerosis (ALS), it is still unknown how they cause this progressive neurodegenerative disease. In a new study, researchers have demonstrated that two ALS-associated genes work in tandem to support the long-term survival of motor neurons.

Common cause of all forms of amyotrophic lateral sclerosis (ALS) discovered

Sun, 21 Aug 2011 14:11:11 EDT

The cause of ALS, a fatal disease that paralyzes its victims, has long eluded scientists. But a new study for the first time has identified a common cause of all forms of ALS, opening a new field to find a treatment. The cause is a broken recycling system in the neurons in the spinal cord and brain that results in severely damaged cells. The finding also may have a wider role in other dementias such as Alzheimer's.

Mutation in SIGMAR1 gene linked to juvenile amyotrophic lateral sclerosis: Sigma-1 receptor offers potential therapeutic target

Fri, 12 Aug 2011 09:15:15 EDT

Researchers have identified a mutation on the SIGMAR1 gene associated with the development of juvenile amyotrophic lateral sclerosis (ALS). Study findings show the gene variant affects Sigma-1 receptors which are involved in motor neuron function and disease development.

Human-cell-derived model of amyotrophic lateral sclerosis provides a new way to study the majority of cases

Thu, 11 Aug 2011 09:48:48 EDT

For decades, scientists have studied a laboratory mouse model that develops signs of the paralyzing disease amyotrophic lateral sclerosis (ALS) as they age. Researchers have now developed a new model of ALS, one that mimics sporadic ALS, which represents about 90 percent of all cases.

Scientists solve mystery of nerve disease genes; Findings may lead to new therapies for Charcot-Marie-Tooth disease and other conditions

Mon, 04 Jul 2011 15:14:14 EDT

For several years, scientists have been pondering a question about a genetic disease called Charcot-Marie-Tooth (CMT) disease type 2D: how can different types of mutations, spread out across a gene, produce the same condition? Now, a team of scientists may have found the answer.

Stem cell model offers clues to cause of inherited ALS

Tue, 21 Jun 2011 15:14:14 EDT

Scientists have used induced pluripotent stem cells derived from patients with amyotrophic lateral sclerosis (ALS) to reveal for the first time how reduced levels of a specific protein may play a central role in causing at least one inherited form of the disease.

Mild obesity appears to improve survival in amyotrophic lateral sclerosis patients

Wed, 11 May 2011 15:13:13 EDT

Patients with amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, may be an exception to the rule that being overweight is a health hazard. In a retrospective study of over 400 ALS patients, researchers found that those who were mildly obese survived longer than patients who were normal weight, underweight or even overweight.

Strides made in understanding amyotrophic lateral sclerosis

Tue, 26 Apr 2011 18:52:52 EDT

Researchers have made a significant advance in the effort to understand amyotrophic lateral sclerosis (ALS) by successfully reversing the toxicity of the mutated protein in the familial type of the disease. Currently there is no cure or prevention for the disease, which affects nerve cells in the brain and the spinal cord. Medical researchers now report success in blocking the lethal effects of the gene by placing several human genes into a yeast cell that shows many similar features to the disease-causing proteins.