Motor neuron diseases News and Research

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USC scientists uncover cellular mechanism responsible for ALS and frontotemporal dementia

Scientists have for the first time discovered a mechanism that limits the number of "cellular janitors" in the nervous system, leading to increased risk for two neurodegenerative diseases: amyotrophic lateral sclerosis and frontotemporal dementia, according to a Keck School of Medicine of USC study published today in Nature Medicine.

7 Feb 2018

Study shows how RNA splicing errors may trigger development of ALS, FTD

The most frequent genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)- rare and related neurological disorders marked by progressive deterioration of motor or cognitive abilities- may be due to errors in RNA splicing, an intermediary step for translating genetic instructions into functional proteins.

14 Jun 2017

Duke researchers discover potential cause for ALS syndromes using mouse models

A team of researchers at Duke University Medical Center led by Dr. Paulo Ferreira and whose work was supported by the National Institutes of Health has found that derailing the transport of certain receptors that carry cargoes from and to the nucleus of motor neurons causes motor neuron disease in mice.

1 Mar 2017

New technology enables “completely locked-in” patients to communicate

A brain─computer interface has enabled people with “locked-in” syndrome to communicate, according to trial results published in PLOS Biology. The device was able to decipher the thoughts of four patients who were unable to speak, move or blink and relay “yes” or “no” answers to basic questions.

2 Feb 2017

Brain-computer interface could revolutionize lives of people with completely locked-in syndrome

A computer interface that can decipher the thoughts of people who are unable to communicate could revolutionize the lives of those living with completely locked-in syndrome, according to a new paper publishing January 31st, 2017 in PLOS Biology.

1 Feb 2017

Scientists build semantic atlas to show how human brain organizes language

What if a map of the brain could help us decode people's inner thoughts? Scientists at the University of California, Berkeley, have taken a step in that direction by building a "semantic atlas" that shows in vivid colors and multiple dimensions how the human brain organizes language. The atlas identifies brain areas that respond to words that have similar meanings.

28 Apr 2016

Cardiac drug combats muscle cramps in ALS

A phase II study shows that the sodium channel blocker mexiletine has no specific safety concerns in patients with amyotrophic lateral sclerosis and, moreover, markedly reduces muscle cramps.

1 Mar 2016

Small peptide TAxI holds promise for carrying biologic drugs into spinal cord

A small peptide dubbed TAxI is living up to its name. Recent studies show it to be an effective vehicle for shuttling functional proteins, such as active enzymes, into the spinal cord after a muscle injection.

17 Feb 2016

Researchers identify protein that could protect against toxic degeneration of cells in ALS

J. Gavin Daigle, a PhD candidate at the LSU Health New Orleans School of Graduate Studies, is the first author of a paper whose findings reveal another piece of the Amyotrophic Lateral Sclerosis (ALS) puzzle.

19 Jan 2016

UCI to receive $8 million from NIH to study brain cell activity in motor neuron disorders

UC Irvine will receive $8 million from the National Institutes of Health to establish one of six national centers dedicated to creating a database of human cellular responses that will accelerate efforts to develop new therapies for many diseases.

9 Oct 2014

New study sheds light on longstanding question about role of mitochondria in motor neuron diseases

A new study by researchers at the University of Utah School of Medicine sheds light on a longstanding question about the role of mitochondria in debilitating and fatal motor neuron diseases and resulted in a new mouse model to study such illnesses.

22 Sep 2014

Human motor neurons can be quickly generated from stem cells, say researchers

Researchers report they can generate human motor neurons from stem cells much more quickly and efficiently than previous methods allowed. The finding, described in Nature Communications, will aid efforts to model human motor neuron development, and to understand and treat spinal cord injuries and motor neuron diseases such as amyotrophic lateral sclerosis (ALS).

2 Apr 2014

Harvard Stem Cell Institute, Evotec Team Up To Take On ALS

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12 Sep 2013

Research collaboration to tackle multiple sclerosis, motor neurone disease

Research into multiple sclerosis and motor neurone disease is to be boosted with an international collaboration to further understanding of these illnesses.

20 Jul 2013

Researchers discover that fruit fly mutation holds clues for treating motor neuron diseases

A team of researchers, led by Marc Freeman, PhD, an early career scientist with the Howard Hughes Medical Institute and associate professor of neurobiology at the University of Massachusetts Medical School have discovered a gene in the fruit fly Drosophila melanogaster that, when mutant, blocks the self-destruction of damaged axons, which could hold clues to treating motor neuron diseases, such as amyotrophic lateral sclerosis.

9 Apr 2013

Researchers identify new mechanism in onset of motor neuron diseases

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.

11 Mar 2013

ARHGEF28 gene mutations present in amyotrophic lateral sclerosis: Research

Researchers at Western University in London, Canada, have identified a new genetic mutation for amyotrophic lateral sclerosis (ALS), opening the door to future targeted therapies. Dr. Michael Strong, a scientist with Western's Robarts Research Institute and Distinguished University Professor in Clinical Neurological Sciences at the Schulich School of Medicine & Dentistry, and colleagues found that mutations within the ARHGEF28 gene are present in ALS.

16 Jan 2013

FUS RNA binding ability essential for development of Amyotrophic Lateral Sclerosis

Research led by Dr. Udai Pandey, Assistant Professor of Genetics at LSU Health Sciences Center New Orleans, has 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.

20 Dec 2012

Biomarker for ALS progression shows promise

Levels of a neurofilament subunit in cerebral spinal fluid, serum, or plasma may predict disease progression in individuals with amyotrophic lateral sclerosis, report researchers.

28 Nov 2012

Molecular link between spinal muscular atrophy and amyotrophic lateral sclerosis

Researchers of motor neuron diseases have long had a hunch that two fatal diseases, amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), might somehow be linked. A new study confirms that this link exists.

28 Sep 2012