Beyond Guam: Cyanobacteria, BMAA and sporadic amyotrophic lateral sclerosis

Institute for Ethnomedicine, Jackson Hole, Wyoming 83001-3464, USA.
Amyotrophic Lateral Sclerosis (Impact Factor: 2.37). 11/2009; 10 Suppl 2(s2):5-6. DOI: 10.3109/17482960903268676
Source: PubMed
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    • "Furthermore, other subpopulations have been found to have a higher prevalence of ALS compared to normal population, namely: some groups of sportsmen, such as Italian football players (Chiò et al., 2005) and American football players (Abel, 2007); and Gulf War Veterans who stayed in Qatar desert (Cox et al., 2009). The common factor of all these events seems to be the exposure to environmental toxins present in football pitches and desert dust, where both cyanobacteria and L-BMAA have been found (Cox et al., 2005; Papapetropoulos, 2007; Bradley and Cox, 2009). Caller et al. (2012) reviewed the literature on important studies of spatial clustering of ALS and explore the hypothesized link between the neurotoxin L-BMAA and ALS. "
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    ABSTRACT: β-N-methylamino-l-alanine (L-BMAA) is a neurotoxic amino acid that has been related to various neurodegenerative diseases. The aim of this work was to analyze the biotoxicity produced by L-BMAA in vivo in rats, trying to elucidate its physiopathological mechanisms and to search for analogies between the found effects and pathologies like Amyotrophic Lateral Sclerosis (ALS). Our data demonstrated that the neurotoxic effects in vivo were dosage-dependent. For evaluating the state of the animals, a neurological evaluation scale was developed as well as a set of functional tests. Ultrastructural cell analysis of spinal motoneurons has revealed alterations both in endoplasmic reticulum and mitochondria. Since GSK3β could play a role in some neuropathological processes, we analyzed the alterations occurring in GSK3β levels in L-BMAA treated rats, we have observed an increase in the active form of GSK3β levels in lumbar spinal cord and motor cerebral cortex. On the other hand, (TAR)-DNA-binding protein 43 (TDP-43) increased in L-BMAA treated animals. Our results indicated that N-acetylaspartate (NAA) declined in animals treated with L-BMAA, and the ratio of N-acetylaspartate/choline (NAA/Cho), N-acetylaspartate/creatine (NAA/Cr) and N-acetylaspartate/choline+creatine (NAA/Cho+Cr) tended to decrease in lumbar spinal cord and motor cortex. This project offers some encouraging results that could help establishing the progress in the development of an animal model of sporadic ALS and L-BMAA could be a useful tool for this purpose.
    Full-text · Article · Apr 2013
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    ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative syndrome which has no known cause, except for a small proportion of cases which are genetically inherited. The development of ALS likely involves both genetic and environmental risk factors. Environmental risk factors implicated in ALS have included heavy metals, trauma, pesticides, electrical injuries, electromagnetic radiation and the cyanobacterial-derived neurotoxin beta-N-methylamino-L-alanine (BMAA). To investigate possible environmental risks, a number of epidemiological studies of ALS have been conducted. Some of these studies employ spatial analysis techniques that examine for spatial clusters of ALS and can help guide further research into identifying environmental exposures. Despite identifying geographical disparities in the distribution of ALS cases, these studies have not provided any clear associations with environmental factors. We review the literature on important studies of spatial clustering of ALS and explore the hypothesized link between the neurotoxin BMAA and ALS.
    Full-text · Article · Jan 2012 · Amyotrophic Lateral Sclerosis
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    ABSTRACT: in animal models such as SOD1 transgenic mice, cell based models, autopsy studies and molecular genetic Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease. The current status of the epidemiology, challenges to its study, and novel study design options are discussed in this paper. We focus on recent results from large-scale population based prospective studies, case-control studies and population based registries, risk factors, and neuropathologic fi ndings in chronic traumatic encephalomyelopathy. We identify areas of interest for future research, including time-trends in the incidence and prevalence of ALS; the meaning of lifetime risk; the phenotypic description of ALS; the defi nition of familial versus sporadic ALS, syndromic aspects of ALS; specifi c risk factors such as military service, life style factors such as smoking, the use of statins, and the presence of β -N-methylamino-L-alanine (BMAA), an excitotoxic amino acid derivative possibly produced by cyanobacteria found in almost every terrestrial and aquatic habitat; the emergence and disappearance of an endemic ALS in areas of the Pacifi c; and gene-environment interactions in the etiology of ALS. To move the epidemiology forward, we suggest using well-characterized cohorts of newly diagnosed ALS patients to identify risk and prognostic factors; storing biological material for future studies; building on the National ALS Registry as a resource of future studies; working in multidisciplinary consortia; and addressing the possible early life etiology of ALS.
    Full-text · Article · Jan 2013 · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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