Discovery Helps Explain Progression of Lou Gehrig's Disease, Offers New Therapy Approach

ScienceDaily (Oct. 17, 2011) — Researchers in Uruguay and Oregon 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.

Even a way to slow its progression would be hugely important, scientists say.

The findings were reported October 17 in Proceedings of the National Academy of Sciences, by researchers from the Pasteur Institute of Montevideo, Clemente Estable Institute and the Linus Pauling Institute at Oregon State University.

The scientists discovered a type of "astrocyte" cell that displays atypical behavior and causes motor neuron death. They are referring to them as aberrant astrocyte, or AbA cells. Astrocyte cells are very common in the brain, and usually help provide metabolic support and protection to neurons. But they can sometimes also become toxic and cause the death of neuron cells.

The researchers now have markers to identify the AbA cell, and found them adjacent to dying motor neuron cells in the spinal cord of laboratory animals with ALS.

The newly-identified AbA cells are selectively toxic to motor neurons, the researchers reported in the study, and 10 times more toxic than any other astrocyte cell known to exist. That level of toxicity is unprecedented, they said.

"We believe these aberrant astrocyte cells are helping drive the progression of ALS," said Joe Beckman, an OSU professor of biochemistry and principal investigator in the Linus Pauling Institute who has been working on amyotrophic lateral sclerosis for more than 15 years.

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New Gene Associated With Amyotrophic Lateral Sclerosis Identified

ScienceDaily (Mar. 3, 2009) — A collaborative research effort spanning nearly a decade between researchers at Massachusetts General Hospital (MGH) and King’s College London (KCL) has identified a novel gene for inherited amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig’s disease). This is the fourth gene associated with familial forms of the devastating neurological disorder.

Two papers, published in the February 27 edition of Science, report mutations in FUS/TLS, a gene known to play a role in DNA repair and the regulation of gene expression. The mutations affect the behavior of the FUS/TLS protein within cells and lead to deposits of abnormal protein within motor neurons.

“We found a series of mutations in a gene that interacts with biological pathways already implicated in ALS and other neurological diseases, resulting in familial ALS of differing inheritance patterns and varying severity,” says Thomas Kwiatkowski, MD, PhD, of the MassGeneral Institute for Neurodegenerative Disease (MGH-MIND), lead author of the MGH report. 

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Gene Find Sheds Light On Motor Neuron Diseases Like ALS

ScienceDaily (Oct. 27, 2008) — Scientists have identified a gene in mice that plays a central role in the proper development of one of the nerve cells that goes bad in amyotrophic lateral sclerosis, or Lou Gehrig's disease, and some other diseases that affect our motor neurons.

The study is the result of a collaboration by scientists at the University of Rochester Medical Center who normally focus on the eye, working together with a developmental neuroscientist at Harvard who focuses on the cerebral cortex. The work appears in the Oct. 23 issue of the journal Neuron.

The work centers on corticospinal neurons, crucial nerve cells that connect the brain to the spinal cord. These neurons degenerate in patients with ALS, and their injury can play a central role in spinal cord injury as well. These are the longest nerves in the central nervous system – nerves sometimes several feet long that run from the brain to the spinal cord. As the ends of the nerves degenerate, patients lose the ability to control their muscles.

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Swamping Bad Cells with Good in ALS Models Helps Sustain Breathing

ScienceDaily (Oct. 20, 2008) — In a disease like ALS - one that's always fatal and that has a long history of research-resistant biology - finding a proof of principle in animal models is significant.

This week, Johns Hopkins researchers report that transplanting a new line of stem cell-like cells into rat models of the disease clearly shifts key signs of neurodegenerative disease in general and ALS in particular - slowing the animals' neuron loss and extending life.

The new work supports the hypothesis that artificially outnumbering unhealthy cells with healthy ones in targeted parts of the spinal cord preserves limb strength and breathing and can increase survival.

An account of the work appears online this week in Nature Neuroscience.

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Lou Gehrig's Protein Found Throughout Brain, Suggesting Effects Beyond Motor Neurons

ScienceDaily (Jun. 16, 2008) —
"Two years ago researchers at the University of Pennsylvania School of Medicine discovered that misfolded proteins called TDP-43 accumulated in the motor areas of the brains of patients with amyotropic lateral sclerosis (ALS), or Lou Gehrig's disease. Now, the same group has shown that TDP-43 accumulates throughout the brain, suggesting ALS has broader neurological effects than previously appreciated and treatments need to take into account more than motor neuron areas."

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