ALS For support and discussion of Amyotrophic lateral sclerosis (ALS), also referred to as "Lou Gehrig's Disease." In memory of BobbyB.


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Old 10-13-2006, 01:05 PM #1
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BobbyB BobbyB is offline
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Join Date: Aug 2006
Location: North Carolina
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BobbyB BobbyB is offline
In Remembrance
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Join Date: Aug 2006
Location: North Carolina
Posts: 4,609
15 yr Member
Post ALS Research News (A monthly summary of significant articles about ALS research)

September 2006

ALS Research News (A monthly summary of significant articles about ALS research)
http://www.catfishchapter.org/news/Sep06Summary.html

Roberta Friedman, Ph.D., ALSA Research Department Information Coordinator

New Protein Identified in FTD Also in ALS
Early Events in ALS Motor Neurons Clarified
Mitochondria Affected by Mutant Protein Linked to Inherited ALS
Loss of Glutamate Control Accelerates ALS in Mice
Cannabinoid Drug Aids Male SOD1 Mutant Mice
Detoxifying Enzymes and the Genetics of ALS
Chinese Do Not Show a VEGF Gene Variation Link to ALS
Abnormal Protein Deposits in ALS Patients
Breathing Support in ALS

While this summary is not exhaustive, it does include some of the most recent advances. If you would like certain news items featured, please contact the Research Department at researchgrants@alsa-national.org.

New Protein Identified in FTD Also in ALS

A protein that is deposited abnormally in both ALS and in the type of dementia that can occur in ALS patients has now been identified in research reported by a team led by Virginia Lee, Ph.D., at the University of Pennsylvania in October in Science. The protein is called TDP-43 and not much is yet known about it, but it will undoubtedly provide a key to the biological process behind both disorders. The protein is labeled by ubiquitin, which marks damaged proteins for destruction. Ubiquitin-labeled deposits were known to be present in both ALS and in the disorder, frontotemporal dementia, which interrupts such cognitive processes as decision making, speech and social functioning. Now that researchers know the identity of the protein that is marked by ubiquitin, they can focus effort on its role in ALS and FTD. The different types of tissue damage evident in the brains of people who died with FTD, reported in October in the American Journal of Pathology by Penn researcher John Trojanowski, M.D., Ph.D., Lee, and collaborators, may now also be explained by investigating further the role of TDP-43.

M Neumann et al. Ubiquinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis. Science DOI: 10.1126/science.1134108 (2006).

Early Events in ALS Motor Neurons Clarified

The earliest events in the demise of motor neurons involve retraction of the nerve endings from muscle but do not immediately activate the process of cell death known as apoptosis. Apparently, changes in mitochondria occur early on in the disease process as modeled by mice that express the mutant protein copper-zinc superoxide dismutase (SOD1). These mice were bred to mice lacking Bax, a protein involved in apoptosis. The resulting SOD1 mice lacking Bax live longer, reported a team led by Ronald Oppenheim, Ph.D., at Wake Forest University in Winston Salem, North Carolina. Their motor neurons show damage but survive despite retracting from muscle. These important clues to how ALS proceeds should give new directions to the search for therapies. For instance, strategies aimed at apoptosis may have to be dropped in favor of addressing the earlier events in motor neuron damage with attention to as yet unknown functions of the Bax protein. The published report appeared in August in the Journal of Neuroscience.

Mitochondria Affected by Mutant Protein Linked to Inherited ALS

Italian researchers led by Maria Teresa Carri, Ph.D., of the Consiglio Nazionale della Ricerche in Rome, collaborating with Joan Valentine, Ph.D., at UCLA, published in the Proceedings of the National Academy of Sciences in September that mutant SOD1 affects the function of the cellular power plants called the mitochondria. For 12 different mutated versions of SOD1, each linked with inherited ALS, all localized more to mitochondria than did the normal protein. All shifted the oxidizing potential of mitochondria as seen in cells growing in lab dishes and damaged the ability of the mitochondria to produce energy. Further evidence that mitochondria are the target of toxic action in ALS will be presented at the coming meeting of the Society for Neuroscience in Atlanta (see concise guide to the Society for Neuroscience meeting) . The ALS Association is funding research toward clarifying the role of mutant SOD1 in mitochondria. Knowing how the mutant protein acts should help in the design of effective therapy. More Info

Loss of Glutamate Control Accelerates ALS in Mice

Nicholas Maragakis, M.D., and colleagues at Johns Hopkins reported in August that mice die a bit sooner from the effects of the SOD1 mutant protein linked to some cases of ALS if they also have reduced action of another protein, GLT1. This protein (the abbreviation stands for glutamate transporter 1) removes excess glutamate, a messenger used by nerve cells and implicated in ALS. Cells that surround and nurture nerve cells use the transporter to sweep up glutamate, which is toxic in excess. The researchers produced mice that express SOD1 and also less than normal amounts of the transporter in spinal cord. These mice have earlier loss of motor neurons and motor function. Findings were published in September in Experimental Neurology.

Cannabinoid Drug Aids Male SOD1 Mutant Mice

Mary Abood, Ph.D., and collaborators at Forbes Norris ALS Center in San Francisco, reported that male mice with the SOD1 mutation showed 12 days delayed loss of motor function if treated with a drug that acts at the body’s own receptors for molecules contained in marijuana. As published in August in the European Journal of Pharmacology, the investigators found that AM1241, a drug available only for researchers, delayed progression to loss of motor function if given either at or after symptoms started. The effect was only seen in male mice, and treatment did not allow the mice to live longer or gain weight. This drug acts at a particular type of cannabinoid receptor, and therefore, does not act in the same way as would marijuana, a complex mixture of many different cannabinoids. It could however serve as a template for designing more effective ALS therapeutics. Other findings on ALS and the body’s own receptors for cannabinoid drugs will be presented at the meeting in October of the Society for Neuroscience in Atlanta (see concise guide to the Society for Neuroscience meeting). More Info

Detoxifying Enzymes and the Genetics of ALS

As covered previously in the July monthly journal news, findings on a possible role in ALS for enzymes that help detoxify nerve gases and pesticides are now published in the September 12 issue of Neurology. People have slightly different versions of protective enzymes, and these genetic differences might help predispose to ALS. The paraoxonase enzymes, abbreviated as PON, detoxify nerve gases and pesticides. These PON enzymes may vary in their ability to efficiently process toxins due to slight differences in the gene that codes their sequence. Now a variation in the PON genes appears to link with susceptibility to ALS. Teams led respectively by Teepu Siddique, M.D., at Northwestern University, Chicago, and Denise Figlewicz, Ph.D., formerly at the University of Michigan, Ann Arbor, and now at the ALS Society of Canada, point out that exposure to such toxins has been suspected for veterans deployed in the Gulf War and that the veterans show increased rates of ALS. In an accompanying editorial, Christopher E. Shaw, M.D., and Ammar Al-Chalabi, M.D., both at King’s College, London, review the known genetics of ALS and note, along with the study investigators, that the disease may result from an interaction of a person’s genetic make-up and their lifetime exposure to possible toxins as well as drug use that can speed up or slow down the action of these protective enzymes. More Info

Chinese Do Not Show a VEGF Gene Variation Link to ALS

Chinese researchers have failed to find a link to ALS among Chinese of a variation in the gene sequence for the protein, vascular endothelial growth factor (VEGF). A link had been reported in some but not all populations investigated. Led by Fan Dongsheng of Peking University Third Hospital, the investigators reported their lack of an association as published in June in Amyotrophic Lateral Sclerosis. Different genetic backgrounds that appear in different geographical locations of people might explain the interaction of genes with environment to make someone more or less likely to get a disease such as ALS, as shown by this and other reports, including one to be presented at the coming meeting of the Society for Neuroscience in Atlanta. This latter report will show that different variants of the gene for angiogenin, also linked to ALS, are associated with the disease in certain parts of the world.

Abnormal Protein Deposits in ALS Patients

Japanese researchers have studied a protein found in the abnormal deposits in nerve tissue of ALS patients. The protein, called p62, is part of the deposited protein found in several degenerative diseases of the nervous system, including ALS. The investigators, led by Koichi Okamoto of Gunma University, found the protein in the spinal cord area where motor neurons die in ALS but not in any deposited protein without the ubiquitin protein tag that helps cells get rid of damaged protein. Control tissue from people without ALS did not show p62 protein. Further study of this protein may reveal a role in the disease process. The report will be published in the Journal of the Neurological Sciences; which appeared on line in July.

Breathing Support in ALS

In the September Neurology, a report and accompanying editorial discuss the benefit of noninvasive breathing assistance (abbreviated as NIPPV) for patients with ALS, especially evident for those who do not have bulbar symptoms, as these patients are able to sleep with the mask for the breathing support. The findings reported by Daniele Lo Coco, M.D., University of Palermo, Italy, and highlighted in the editorial by Terry Heiman-Patterson, M.D., Drexel University, Philadelphia, and Robert Miller, MD, California Pacific Medical Center, San Francisco, show better quality of life and longer survival of ALS patients without severe bulbar symptoms who used NIPPV. A long term NIH funded trial will be examining the benefit of breathing assistance in ALS in detail.

A review of patient records showed that ALS patients who used breathing support had better sleep quality and survived longer. Either noninvasive breathing aids or tracheostomy produced improvements compared to patients not using breathing assistance. Findings were reported by Linda Schachter, Ph.D., and colleagues at the Institute for Breathing and Sleep, Victoria, Australia, in June in Amyotrophic Lateral Sclerosis.

The nighttime impairment of oxygenation due to breathing weakness in ALS is not detected by usual clinical means, another group of Australian investigators led by Dominic Rowe, Ph.D., of the Royal North Shore Hospital, Sydney, reported in July in the Internal Medicine Journal.

Columbia University researchers led by Hiroshi Mitsumoto, M.D., published in June in Amyotrophic Lateral Sclerosis findings on ALS patients who chose to live with breathing sustained by a ventilator machine. On average they were younger, had young children, were better educated, and of more financial means than those not choosing long term mechanical ventilation. They also appeared more optimistic and none were clinically depressed, as compared to those declining this approach, a quarter of whom scored as depressed. Half of those declining ventilation had mild cognitive symptoms compared to 14 percent of those choosing it. The choice can be explored by families and their clinicians, the researchers concluded.
http://www.catfishchapter.org/news/Sep06Summary.html
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