Abstract
There is mounting evidence to suggest that environmental factors play a major role in the development of neurodegenerative diseases like ALS (Amyotrophic Lateral Sclerosis). The non-protein amino acid beta-N-methylamino-L-alanine (BMAA) was first associated with the high incidence of Amyotrophic Lateral Sclerosis/Parkinsonism Dementia Complex (ALS/PDC) in Guam, and has been implicated as a potential environmental factor in ALS, Alzheimer’s disease, and other neurodegenerative diseases. BMAA has a number of toxic effects on motor neurons including direct agonist action on NMDA and AMPA receptors, induction of oxidative stress, and depletion of glutathione. As a non-protein amino acid, there is also the strong possibility that BMAA could cause intraneuronal protein misfolding, the hallmark of neurodegeneration. While an animal model for BMAA-induced ALS is lacking, there is substantial evidence to support a link between this toxin and ALS. The ramifications of discovering an environmental trigger for ALS are enormous. In this article, we discuss the history, ecology, pharmacology and clinical ramifications of this ubiquitous, cyanobacteria-derived toxin.
Highlights
Amyotrophic Lateral Sclerosis (ALS) is a debilitating and fatal neuromuscular disease with an average annual incidence worldwide of 2 per 100,000
BMAA is mainly concentrated in proteins and was consumed by Chamorro through multiple dietary sources, including cycad flour, flying foxes, and other animals that fed on cycad seeds [42,44,45,46]
They were able to demonstrate that consumption of cycad flour, flying foxes, and other animals that fed on cycad seeds by the indigenous Chamorro people led to bioconcentration of protein-bound BMAA up the food chain, leading to the accumulation of BMAA in the brains of Chamorro patients with Amyotrophic Lateral Sclerosis/Parkinsonism Dementia Complex (ALS/PDC) [34,42,44,45,46,47]
Summary
Amyotrophic Lateral Sclerosis (ALS) is a debilitating and fatal neuromuscular disease with an average annual incidence worldwide of 2 per 100,000. Of ALS cases occur sporadically, with no known familial history. At present there is no known cause for sporadic ALS, though a number of environmental compounds have been implicated as potential etiological agents. Possible clusters of ALS have been described amongst soccer players sharing the same environmental risks and occupational activities including frequent head trauma [18,19,20,21,22,23,24], but recent reports have refuted specific links of head trauma to ALS [25,26,27]. Of the environmental triggers for ALS, the cyanobacteria-derived neurotoxin BMAA continues to attract attention because of its ability to cause neurodegeneration in vitro and its ubiquitous nature [29,30,31,32,33], providing support for a potential role in the etiology of sporadic ALS [29,34,35]
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