Nieuws amyotrophic lateral sclerosis


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Blootstelling aan pesticiden kan risicofactor zijn voor ALS

Nieuw onderzoek toont aan dat iemand door milieuvervuilers meer kans zou kunnen hebben op de ontwikkeling van amyotrofische laterale sclerose, of ALS. Er is geen behandeling voor deze progressieve zenuw- en spierziekte, ook bekend als de ziekte van Lou Gerigh. Mensen met deze ziekte verliezen uiteindelijk alle kracht en mogelijkheden om hun armen, benen en lichaam te bewegen.

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Het eten van helder gekleurde groenten en fruit kan ALS vertragen of zelfs voorkomen

Nieuw onderzoek suggereert indien men meer levensmiddelen consumeert die kleurrijke carotenoïden bevatten, met name bèta-caroteen en luteïne, het begin van amyotrophic lateral sclerosis (ALS) kan vertragen of voorkomen. De studie, gepubliceerd door Wiley in “Annals of Neurology, een dagboek van de Amerikaanse Neurologische Vereniging en Kind Neurologie ondervonden dat een diët vol met Lycopeen (Lycopeen is een helderrood carotenoïde dat in tomaat en andere rode vruchten, zoals watermeloen en rozebottels, zit. Beta Kryptoxanthine (Kryptoxanthine is een natuurlijke rode kleurstof. Ze komt voor in bloemen en wortels van planten van de soort Physalis, maar ook in sinaasappelschil, papaja, eierdooier, boter en in serum van runderbloed. Het is een antioxidant en kan dus helpen om kanker te voorkomen. en vitamine C, het ALS risico niet deed verminderen. Carotenoïden geven groenten en fruit hun fel oranje, rode of gele kleuren, en zijn een bron van vitamine A. Voorafgaande onderzoeken lieten zien dat oxidatieve stress een grote rol speelt in de ontwikkeling van ALS.

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Vergiftiging door industriële componenten kunnen overeenkomstige effecten veroorzaken als ALS

Een onderzoek door researchers aan IDIBELL-UB relateert vergiftiging door IDPN nitriel aan de symptomen van amyotrope laterale sclerose. Researchers van de Bellvitge Biomedical Research Institute (IDIBELL) aan de Universiteit van Barcelona (UB) hebben een onderzoek gecoördineerd in hoe de IDPN nitriel neurologische syndromen veroorzaakt die gelijk zijn aan die van de amyotrope laterale sclerose (ALS), een ernstige neurologische degeneratieve aandoening.

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TV - Tegenlicht

Documentaire. Op 6 augustus dit jaar overleed de Britse historicus Tony Judt op 62-jarige leeftijd aan de gevolgen van de spierziekte ALS. Judt was een groot denker over het politieke landschap waarin we leven. Zijn testament was de magistrale lezing 'What is Living and What is Dead in Social Democracy?' gehouden in New York vorig jaar. Hierin pleitte hij er op een aangrijpende manier voor om nu meer dan ooit de sociaal-democratie te herdenken. Zijn pleidooi klinkt door in drie portretten van hedendaagse verliezers van het door Judt gehekelde marktdenken: John Gerrits uit Sittard zag het buurthuis, waar hij dertig jaar sociaal werker was, verdwijnen omdat de gemeente met die grond liever winst maakte. Mark Goossens werkte ruim vijftien jaar in de Antwerpse Opel-fabriek, die in december definitief de deuren zal sluiten. En Laurent Giacomelli verloor zijn werk en zijn gezondheid als gevolg van de privatisering van France Telecom.

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Onderzoekers ontdekken genetische link tussen beide types van ALS

Onderzoekers van de Northwestern University Feinberg School of Medicine hebben een verband ontdekt tussen sporadische en familiale vormen van amyotrofische lateraal sclerose (ALS), een neurodegeneratieve ziekteook wel bekend als Lou Gehrig's ziekte. Onderzoekers verbonden een zeldzame genetische oorzaak voor de meeste gevallen van ALS, de weg vrijmaakt voor therapie gebaseerd opeen bekend moleculair doelwit.

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John Huizinga


Ontwikkeling Leuvens kandidaat-medicijn tegen zenuwziekte ALS
geraakt in hogere versnelling

Het Europees Geneesmiddelmiddelenbureau EMEA heeft een Leuvens kandidaat-medicijn tegen de zenuwziekte ALS (Amyotrofische Laterale Sclerose) erkend als een 'weesgeneesmiddel'. Dat is een titel voorbestemd voor veelbelovende stoffen, die zonder extra financiële en administratieve stimulans niet op de markt zouden geraken. Het kandidaat-geneesmiddel is gebaseerd op het werk van wetenschapper Peter Carmeliet van VIB (Vlaams Instituut voor Biotechnologie) en de K.U.Leuven. Carmeliet ontdekte dat een tekort aan een bepaald eiwit (VEGF) symptomen van ALS veroorzaakt bij muizen. Het huidig onderzoek spitst zich toe op het rechtstreeks toedienen van VEGF in de hersenen van patiënten, via een pompje. Dat zou het verloop van de ziekte moeten afremmen, een effect dat in eerder VIB-onderzoek is waargenomen bij proefdieren. De ontwikkeling van de therapie gebeurt door het farmaceutisch bedrijf NeuroNova. De klinische testen lopen in het UZ Leuven, onder leiding van Wim Robberecht, eveneens verbonden aan VIB en het Vesalius Research Center van de K.U.Leuven.

Exclusieve toegang tot de markt
Met de regeling inzake weesgeneesmiddelen wil de Europese Unie stimuleren dat ook voor zeldzamere ziekten geneesmiddelen worden ontwikkeld. Zonder die steun is de economische haalbaarheid van nieuwe geneesmiddelen vaak te onzeker. De erkenning als weesgeneesmiddel levert de producent 10 jaar marktexclusiviteit op, net zoals centrale registratie in de Europese Unie, advies bij opstellen van onderzoeksprotocollen en registratieaanvragen en reductie van registratiekosten voor het nieuwe middel. Ook kan het onderzoek gebeuren met minder patiënten. Zeker bij ziektes die erg zeldzaam zijn, kan dat de ontwikkeling van een geneesmiddel gevoelig versnellen.

Ongeneeslijke en verlammende aandoening
ALS is een ongeneeslijke verlammende aandoening, met astrofysicus Stephen Hawking als één van de meest iconische patiënten. In België lijden ongeveer 800 à 1.000 mensen aan de ziekte. Jaarlijks overlijden er meer dan 200 patiënten en komen er minstens evenveel bij. Bij patiënten treedt er aftakeling op van de zenuwbanen die naar de spieren lopen. Daardoor verliezen de patiënten de controle over hun spieren en geraken ze volledig verlamd, terwijl ze blijven beschikken over hun mentale vermogens.

Lange weg tot geneesmiddel
Alhoewel de erkenning als weesgeneesmiddel het onderzoek in een stroomversnelling zal brengen, blijft de weg naar VEGF als een geregistreerd geneesmiddel weg nog lang. Gereglementeerde studies zullen het therapeutisch effect van VEGF om de ontwikkeling van ALS te remmen nog moeten aantonen. Het huidig onderzoek bevindt zich in klinische fase I/II. Dat betekent dat de veiligheid nu bij een gecontroleerde groep van 200 patiënten zal worden geëvalueerd.


Treating Lou Gehrig's Disease

More than 300,000 Americans alive today will eventually die of ALS, which is more commonly known as Lou Gehrig's disease. Katie Couric reports on better understanding this mysterious disease.

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Leaky blood vessels open up nerve cells to toxic assault in Lou Gehrig's disease

Leaky blood vessels that lose their ability to protect the spinal cord from toxins may play a role in the development of amyotrophic lateral sclerosis, better known as ALS or Lou Gehrig's disease, according to research published in the April issue of Nature Neuroscience.

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Penn researchers find potential in yeast for selecting Lou Gehrig's disease drugs

Researchers from the University of Pennsylvania School of Medicine are developing a novel approach to screen for drugs to combat neurodegenerative diseases such as amyotrophic lateral sclerosis, or Lou Gehrig's disease, using yeast cells.

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Protecting neurons could halt Alzheimer's, Parkinson's diseases

Researchers at Southern Methodist University (SMU) and The University of Texas at Dallas (UTD) have identified a group of chemical compounds that slow the degeneration of neurons, a condition behind old-age diseases like Alzheimer's, Parkinson's and amyotrophic lateral sclerosis (ALS). Their findings are featured in the November 2008 edition of Experimental Biology and Medicine. SMU Chemistry Professor Edward R. Biehl and UTD Biology Professor Santosh D'Mello teamed to test 45 chemical compounds. Four were found to be the most potent protectors of neurons, the cells that are core components of the human brain, spinal cord and peripheral nerves.The most common cause of neurodegenerative disease is aging. Current medications only alleviate the symptoms but do not affect the underlying cause – degeneration of neurons. The identification of compounds that inhibit neuronal death is of urgent and critical importance.

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New study finds blood-spinal cord barrier compromised in mice with ALS

The blood-spinal cord barrier is functionally impaired in areas of motor neuron damage in mice modeling amyotrophic lateral sclerosis, report researchers at the University of South Florida Center for Aging and Brain Repair. The barrier disruption was found in mice at both early and late stages of ALS, a progressive neurodegenerative disease affecting nerve cells in the brain and the spinal cord. The study appears in the online, open-access journal PLoS ONE.

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Gene newly linked to inherited ALS may also play role in common dementia

Scientists at Washington University School of Medicine in St. Louis have linked a mutation in a gene known as TDP-43 to an inherited form of amyotrophic lateral sclerosis, the neurodegenerative condition often called Lou Gehrig's disease.

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ALS Aggregates Are Composed of Only One Protein

Researchers have provided a big new clue to help combat amyotrophic lateral sclerosis (ALS), deciphering that the dense protein aggregates that contribute to the nerve decay of ALS are composed of just one protein: superoxide dismutase (SOD1).

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One Darn Good Reason To Avoid Cholesterol Lowering Drugs

The national average of those who suffer from ALS is a mere .0005 percent. But – sit down for this one – among those who reported suffering from “drug induced ALS,” nearly a third were using cholesterol lowering drugs! Apparently, this class of drugs can “tangle” a protein known as “tau proteins.”

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Hope among patients with ALS may take a variety of forms

Sustaining hope in the face of a chronic, debilitating illness such as amyotrophic lateral sclerosis should be a goal of palliative care and can take many forms, representing a continuum from focusing on the self to concern for others, as described in a paper published in the April issue of Journal of Palliative Medicine, a peer-reviewed publication of Mary Ann Liebert, Inc.

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Caffeine Appears To Be Beneficial In Males–But Not Females–With Lou Gehrig’s Disease

Amyotrophic lateral sclerosis (ALS) is a fatal disease that damages key neurons in the brain and spinal cord. The disease causes progressive paralysis of voluntary muscles and often death within five years of symptoms. Although ALS (Lou Gehrig’s disease) was discovered over a century ago, neither the cause nor a cure have been found, but several mechanisms seem to play a role in its development, including oxidative stress. Researchers agree that ALS is a multifactorial disease that involves a complex interplay between a genetic predisposition and environmental factors. One environmental factor is diet. With oxidative stress (which damages the cells) a common concern in ALS pathology, it is worth examining what role antioxidants (which confer benefits to the cells) might play. Antioxidants (the vitamins and nutrients that protect the cells from damage) are found in commonly consumed beverages and foods. Coffee in particular has received attention as a potent dietary antioxidant. It is worth noting that coffee has significantly more antioxidant capacity than cocoa and green, black or herbal teas. However, coffee contains several components, the largest of which are caffeine and chlorogenic acid, a dietary polyphenol that is beneficial to the immune system. Previous studies have shown positive effects with coffee, caffeine, or chlorogenic acid supplementation in improving oxidative stress and its associated cell death mechanisms.

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Spinal fluid proteins signal Lou Gehrig's disease

High levels of certain proteins in the spinal fluid could signal the onset of Lou Gehrig's disease, according to researchers. The discovery of these biomarkers may lead to diagnostic kits for early diagnosis, accurately measuring the progression of the disease and monitoring the effects of treatment. Lou Gehrig's disease -- or Amyotrophic Lateral Sclerosis (ALS) -- is caused by the degeneration of nerve cells controlling the voluntary movement of muscles. However, it is hard to diagnose because symptoms such as muscle weakness are common in other ailments and currently, there is no diagnostic test for the disease. "The disease has to progress far enough so that the patient begins to experience significant muscle weakness, so that a physician can identify the problem," said James Connor, distinguished professor and vice-chair of neurosurgery, Penn State Hershey. "If we had a biomarker we could start treatments earlier and perhaps save more nerve cells and slow the disease." The problem is compounded by the speed at which the disease progresses. In some patients the disease can run its course in just a couple of years, while in others it can take seven to ten years. To find an early warning signal for the onset of Lou Gehrig's disease, Connor and his colleagues, Zachary Simmons and Ryan Mitchell at the Hershey Medical Center, focused their attention on proteins related to inflammation in the spinal cord. Studies show that the progression of the disease involves excessive inflammation of nerve cells. The team also argued that because these proteins tend to be much smaller than most other proteins, they are likely to be overlooked in large-scale protein studies. The researchers extracted spinal fluid from two groups of patients. The first group, comprising 41 patients, was known to have Lou Gehrig's disease, while the second group of 31 patients complained of muscle problems such as weakness and cramps.

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UI team identifies genes that improve survival in mice with ALS

University of Iowa researchers investigating the basic biology of cell signaling have made a discovery that may have therapeutic implications for amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. The UI team, led by John Engelhardt, Ph.D., professor and head of anatomy and cell biology in the UI Roy J. and Lucille A. Carver College of Medicine, discovered that two cell-signaling proteins called Nox1 and Nox2 appear to play an important role in disease progression of an inherited form of ALS. This work is published in the Sept. 13 issue of the Journal of Clinical Investigation.Deleting either Nox1 or Nox2 genes from mice with the inherited type of ALS significantly increased the lifespan of the mice. Nox2 deletion produces the most dramatic effect, nearly doubling the lifespan of the mice. In addition, Nox2 deletion dramatically increased the survival index -- the time from disease onset to death. This is the first report of a single gene that affects the survival index in ALS models.

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NH lake linked to ALS cases

The risk of developing a fatal neurodegenerative disease is 25 times higher than the norm for people who live around Mascoma Lake, according to researchers studying the possibility of a link between lake bacteria and neurological illness.

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Gene find sheds light on motor neuron diseases like ALS

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. The team led by Lin Gan, Ph.D., of Rochester and Jeffrey D. Macklis, M.D., D.HST, of Harvard showed that a protein known as Bhlhb5 is central to how the brain's progenitor cells ultimately become corticospinal motor neurons, one type of neuron that deteriorates in ALS. The same group of neurons also degenerates in patients with a rare neurological disease known as hereditary spastic paraplegia. The work by the Harvard and Rochester scientists marks an important step in scientists' understanding of how stem cells in the brain eventually grow into the extraordinary network of circuits that make up the human nervous system. Understanding how the body determines the destiny of stem and progenitor cells is crucial if physicians are to ultimately use the cells to create new treatments for motor neuron diseases like ALS and HSP, as well as other conditions such as Parkinson's and Huntington's diseases and spinal cord injury. Macklis' team is a world leader in discovering how the brain determines the destiny of its cells. The process is a bit like what happens on a construction site, where a foreman taps the expertise of a variety of workers – carpenters, plumbers, bricklayers, and so on – as needed to build a given structure. In the brain, teams of molecular signaling molecules are brought together to create nerve cells out of raw material where and when needed. Hundreds of such signaling molecules are brought together instantly and continually to allow the brain to create the nerve cells it needs for growth and development. "How does the brain take a broad class of neurons and decide which ones to send to the spinal cord, or which will connect to our visual centers?" said Macklis, who is director of the Center for Nervous System Repair at Massachusetts General Hospital and at Harvard.

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Human stem cells provide a new model for Lou Gehrig's disease

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a devastating condition in which motor neuron degeneration causes progressive loss of movement and muscle tone, leading to death. Overcoming the limited success of previous models, a report published in Disease Models & Mechanisms (DMM), dmm.biologists.org describes how neurons can be derived from human stem cells, and engineered to mimic inherited ALS. Researchers at the University of California Los Angeles developed an optimized protocol to generate motor neurons from human embryonic stem cells (ES cells), which express normal or mutant forms of the SOD-1 gene, which is linked to inherited, familial ALS. Resulting cells exhibit hallmark characteristics of motor nerve cells, and neurons expressing mutant SOD-1 display abnormalities typical of ALS. Defects included shortened cell projections and a reduced life span compared to cells containing the normal SOD-1 gene. This human cell-derived model of ALS provides a new method of studying this disease and testing novel therapeutics. This is especially helpful as only one drug is approved to help slow ALS progression, and animal models currently used in drug development have had limited success. Additionally, this research may aid other gene-linked neurodegenerative diseases, as they too may benefit from studies in a human cell-derived model.

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Researchers identify ALS gene mutation

Research that has discovered a new gene whose mutations cause 5 percent of inherited cases of ALS (amyotrophic lateral sclerosis) is part of a national study led by the Northwestern University Feinberg School of Medicine. The study reported in Science today (Feb. 27) points to a common cellular deficiency in the fatal neurological disorder, said Teepu Siddique, M.D., Les Turner ALS Foundation/Herbert C. Wenske Foundation Professor in the Davee Department of Neurology and Clinical Neurosciences and Department of Cell and Molecular Biology and Director of the Division of Neuromuscular Medicine at the Feinberg School. The new research is part of a national collaboration directed by Siddique, the principal investigator for the "Genetics of ALS" project funded at Feinberg by the National Institutes of Health. Earlier research by Siddique and colleagues extended the genetic knowledge of familial (inherited) ALS by identifying the first and second ALS genes (the SOD1 gene in 1993 and the ALSIN gene in 2001), in addition to identifying loci on chromosomes 9, 15, 16, and X. The study published today discovered aFUS/TLS gene mutations in ALS families collected through efforts of the NIH-funded multi-center project and included among others a large Italian family previously studied by Siddique and Cortelli.

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UMMS researchers isolate first 'neuroprotective' gene in patients with amyotrophic lateral sclerosis

A genetic variant that substantially improves survival of individuals with amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, has been indentified by a consortium of researchers led by John Landers, PhD, Associate Professor of Neurology and Robert Brown, MD, DPhil, Chair and Professor of Neurology at the University of Massachusetts Medical School. Discovery of the KIFAP3 gene variant is reported in the Proceedings of the National Academy of Sciences. "This report is the first to describe genetic factors that determine rate of progression in ALS," said Brown. "The finding reflects a truly international collaboration in which physicians and scientists from nearly 20 teams in several countries worked together to use new methods in genetics to understand ALS." ALS is a progressive, neurodegenerative disorder affecting the motor neurons in the central nervous system. As motor neurons die, the brain's ability to send signals to the body's muscles is compromised. This leads to loss of voluntary muscle movement, paralysis and eventually death from respiratory failure. In 1993, a team of researchers led by Dr. Brown discovered the first gene linked to familial ALS, a protein anti-oxidant known as superoxide dismutase, or SOD1. Earlier this year, Dr. Brown and his colleagues discovered a mutation in the FUS/TLS gene which is estimated to account for 5 percent of inherited ALS cases. There are only four genes known, that when mutated, cause familial ALS. The KIFAP3 gene variant is the first to be linked with the rate of progression in ALS. To isolate the KIFAP3 gene variant, a consortium of researchers from the U.S., Mexico, Israel and Europe examined more than 300,000 genetic variants in over 1,800 people with ALS and nearly 2,200 unaffected controls. The approach is based on the assumption that naturally occurring gene variations can influence both disease susceptibility and the way a disease runs its course once underway. During their search, the consortium detected a beneficial variant of the KIFAP3 gene which was associated with an increase in survival time of 40 to 50 percent.

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Researchers identify a gene responsible for cases of Lou Gehrig’s disease

A team of Canadian and French researchers has identified a novel gene responsible for a significant fraction of ALS (sporadic amyotrophic lateral sclerosis) cases. ALS is commonly referred to as Lou Gehrig’s disease, an incurable neuromuscular disorder that affects motor neurons and leads to paralysis and death within one to five years. Published in the current online edition of Nature Genetics, the study on 200 human subjects with ALS was led by Doctors Guy Rouleau, Edor Kabashi, Paul Valdmanis of the Research Centre of the Centre hospitalier de l'Université de Montréal (CRCHUM). The team identified several genetic mutations in the TDP-43 gene by studying ALS patients from France and Quebec. They established TDP-43 as the gene responsible for up to five percent of the ALS patients. The breakthrough is the result of teamwork with peers from the Waterloo and Laval universities in Canada and the Fédération des maladies du système nerveux and the Institute of Biology (Unité de Neurologie Comportementale et Dégénérative) in France.

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More genes for Lou Gehrig's disease identified, according to Penn researchers

In recent months a spate of mutations have been found in a disease protein called TDP-43 that is implicated in two neurodegenerative disorders: amyotrophic lateral sclerosis, also called Lou Gehrig’s disease, and certain types of frontotemporal dementia. These mutations could potentially become candidates for drug targets. Recently, colleagues at the University of Pennsylvania School of Medicine and Veterans Affairs in Seattle, Wash. have found two more mutations.

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als
Chemical Exposure May Increase Risk of ALS

Preliminary results show that a common environmental chemical may increase the risk of developing amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, according to research that will be presented at the American Academy of Neurology 60th Anniversary Annual Meeting in Chicago, April 12–19, 2008.

The study was based on the Cancer Prevention Study II of the American Cancer Society. Over one million people were asked to report their exposure to 12 types of chemicals. The participants were followed for 15 years, and the number of people who died during that time of ALS was tracked. A total of 617 men and 539 women died from ALS during the study.

Researchers found no significant link between ALS and exposure to most chemicals, including pesticides and herbicides. People who reported that they had regular exposure to formaldehyde, however, were 34 percent more likely to develop ALS than those with no exposure to formaldehyde.

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This was the first website dedicated specifically to ALS/MND

It explores if a combination of existing medications and supplements, plus lifestyle and dietary changes may slow the progress of neurodegeneration in ALS/MND. I was diagnosed with ALS/MND in February 1994 but am now in remission. I am still walking, talking and looking after myself. My symptoms decreased significantly and have not worsened in eight years. Why this is the case is explained on this website. It may be due to my experimentation with antioxidants, diet and lifestyle changes. Other factors
are almost certainly involved and are also discussed here.

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Gerrit de Jong


Discovery of active genes reveals new clues on ALS

A University of California, Irvine neurologist is part of a national group of scientists who have identified the active genes in sporadic amyotrophic lateral sclerosis, a discovery that provides expanded opportunities for developing therapies to treat

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New step in DNA damage response in neurons discovered

Researchers have identified a biochemical switch required for nerve cells to respond to DNA damage. The finding, scheduled for advance online publication in Nature Cell Biology, illuminates a connection between proteins involved in neurodegenerative disease and in cells' response to DNA damage. Most children with the inherited disease ataxia telangiectasia are wheelchair-bound by age 10 because of neurological problems. Patients also have weakened immune systems and more frequent leukemias, and are more sensitive to radiation. The underlying problem comes from mutations in the ATM (ataxia telangiectasia mutated) gene, which encodes an enzyme that controls cells' response to and repair of DNA damage. ATM can be turned on experimentally by treating cells with chemicals that damage DNA. After other proteins in the cell detected broken DNA needing repair, scientists had thought that the ATM protein could activate itself directly. Emory researchers have shown that an additional step is necessary first. "In neurons that are not dividing anymore, we now know that another regulator is involved: Cdk5," says Zixu Mao, MD, PhD, associate professor of pharmacology and neurology at Emory University School of Medicine. Working with postdoctoral fellows Bo Tian, PhD and Qian Yang, PhD, Mao found that the Cdk5 protein must activate ATM before ATM can do its job in neurons. The results support the idea that Cdk5 may be a potential drug target. Cdk5 contributes to normal brain development, and aberrant Cdk5 activity is known to be involved in the death of neurons in several neurodegenerative diseases, including Alzheimer's, Parkinson's and amyotrophic lateral sclerosis.

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Researchers genetically link Lou Gehrig's disease in humans to dog disease

An incurable, paralyzing disease in humans is now genetically linked to a similar disease in dogs. Researchers from the University of Missouri and the Broad Institute have found that the genetic mutation responsible for degenerative myelopathy (DM) in dogs is the same mutation that causes amyotrophic lateral sclerosis (ALS), the human disease also known as Lou Gehrig's Disease. As a result of the discovery, which will be published in the Proceedings of the National Academy of Sciences this week, researchers can now use dogs with DM as animal models to help identify therapeutic interventions for curing the human disease, ALS. "We uncovered the genetic mutation of degenerative myelopathy, which has been unknown for 30 years, and linked it to ALS, a human disease that has no cure," said Joan Coates, a veterinary neurologist and associate professor of veterinary medicine and surgery in the MU College of Veterinary Medicine. "Dogs with DM are likely to provide scientists with a more reliable animal model for ALS. Also, this discovery will pave the way for DNA tests that will aid dog breeders in avoiding DM in the future." Previously, ALS research has relied heavily on transgenic rodents that expressed the mutant human gene SOD1, which causes ALS. Researchers found that dogs with DM also had mutations in their SOD1 gene. Many rodent models possess very high levels of the SOD1 protein that can produce pathologic processes distinct from those occurring in ALS patients. Since the SOD1 mutation is spontaneous in dogs, the clinical spectrum in dogs may represent more accurately that of human ALS. "Compared with the rodent models for ALS, dogs with DM are more similar to people in size, structure and complexity of their nervous systems, and duration of the disease," said Gary Johnson, associate professor of veterinary pathobiology in the MU College of Veterinary Medicine. "The results from clinical trials conducted with DM-affected dogs may better predict the efficacies of therapeutic interventions for treating ALS in humans."

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Protein plays Jekyll and Hyde role in Lou Gehrig's disease

Amyotrophic lateral sclerosis, more commonly known as Lou Gehrig's disease, is a fatal neurodegenerative disease caused by the death of motor neurons in the brain and spinal cord that control muscle movements from walking and swallowing to breathing. In a groundbreaking study this week in PLoS Biology, Brandeis and Harvard Medical School scientists report key findings about the cause and occurrence of the familial form of ALS.

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Harvard-Columbia team creates neurons from ALS patient's skin cells

Harvard and Columbia scientists have for the first time used a new technique to transform an ALS patient's skin cells into motor neurons, a process that may be used in the future to create tailor-made cells to treat the debilitating disease. The research will be published July 31 in the online version of the journal Science.

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Data mining detects signs of Lou Gehrig's disease in gene carriers long before symptoms appear

Inspired by the use of microarray chips that look for gene combinations, psychologists are using "pattern array" software to spot movements in rats that might help them predict diseases such as Lou Gehrig's syndrome.

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Targeting astrocytes slows disease progression in ALS

In what the researchers say could be promising news in the quest to find a therapy to slow the progression of amyotrophic lateral sclerosis, or Lou Gehrig's disease, scientists at the University of California, San Diego School of Medicine have shown that targeting neuronal support cells called astrocytes sharply slows disease progression in mice.

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Data Mining Detects Signs of Lou Gehrig's Disease in Gene Carriers Long Before Symptoms Appear

Inspired by the use of microarray chips that look for gene combinations, psychologists are using "pattern array" software to spot movements in rats that might help them predict diseases such as Lou Gehrig's syndrome. A report in the August issue of Behavioral Neuroscience, published by the American Psychological Association, describes how this novel use of data mining may enable investigators to test therapies to delay or even prevent disease, starting with hereditary forms.

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First trial in patients with a potential treatment of the incurable ALS muscle disease - Project of VIB, UZ Leuven and NeuroNova

Permission has been granted to start the first safety and tolerability trial on patients for a remedy for ALS. ALS is an incurable, paralyzing neurodegenerative disorder that strikes 5 persons in every 100,000. The disease commonly affects healthy people in the most active period of their lives - without warning. Researchers from VIB at the K.U.Leuven have previously shown the possibilities for the use of VEGF in the treatment of ALS through work in animal models. The Swedish Biopharmaceutical company NeuroNova has already built upon this research. Together with UZ Leuven they’ll start the first evaluation of safety and tolerability of the drug in patients by the end of this year. This is an important step in the development of a new treatment. It will take several years before the protein can be made available as a medicine.

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A novel human stem cell-based model of ALS opens doors for rapid drug screening

Long thought of as mere bystanders, astrocytes are crucial for the survival and well-being of motor neurons, which control voluntary muscle movements. In fact, defective astrocytes can lay waste to motor neurons and are the main suspects in the muscle-wasting disease amyotrophic lateral sclerosis (ALS). To get to the root of this complicated relationship, researchers from the Salk Institute for Biological Studies for the very first time established a human embryonic stem cell (hESC)-based system for modeling ALS. Their study confirmed that dysfunctional human astrocytes turn against their charges and kill off healthy motor neurons. But more importantly, treating the cultured cells with apocynin, a powerful anti-oxidant, staved off motor neuron death caused by malfunctioning astrocytes.Their findings, which appear in the Dec. 4 issue of the journal Cell Stem Cell, provide new insight into the toxic pathways that contribute to the demise of motor neurons in ALS and open up new possibilities for drug-screening experiments using human ALS in vitro models, as well as clinical interventions using astrocyte-based cell therapies. "A variety of drugs that had demonstrated significant efficacy in mouse models didn't keep their promise in both preclinical and clinical trials," says Fred H. Gage, Ph.D., a professor in the Laboratory for Genetics, who led the study. In fact, just one drug—riluzole— has been approved by the FDA to treat ALS, and it only slows the course of the disease by two months. "There is an urgent need for new ALS models that have the potential to translate into clinical trials and that could, at a minimum, be used in conjunction with the murine models to verify drugs and drug targets," says Gage.

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