Abstract
A cluster of amyotrophic lateral sclerosis (ALS) has been previously described to border Lake Mascoma in Enfield, NH, with an incidence of ALS approximating 25 times expected. We hypothesize a possible association with cyanobacterial blooms that can produce β-N-methylamino-l-alanine (BMAA), a neurotoxic amino acid implicated as a possible cause of ALS/PDC in Guam. Muscle, liver, and brain tissue samples from a Lake Mascoma carp, as well as filtered aerosol samples, were analyzed for microcystins (MC), free and protein-bound BMAA, and the BMAA isomers 2,4-diaminobutyric acid (DAB) and N-(2-aminoethyl)glycine (AEG). In carp brain, BMAA and DAB concentrations were 0.043 μg/g ± 0.02 SD and 0.01 μg/g ± 0.002 SD respectively. In carp liver and muscle, the BMAA concentrations were 1.28 μg/g and 1.27 μg/g respectively, and DAB was not detected. BMAA was detected in the air filters, as were the isomers DAB and AEG. These results demonstrate that a putative cause for ALS, BMAA, exists in an environment that has a documented cluster of ALS. Although cause and effect have not been demonstrated, our observations and measurements strengthen the association.
Highlights
It is probable that one or more environmental toxins contribute to the etiology of sporadicAmyotrophic Lateral Sclerosis (ALS), most likely interacting with underlying genetic susceptibility factors [1,2]
In 2009, we described a number of cases of amyotrophic lateral sclerosis (ALS) from Enfield, NH
BMAA was identified within the Lake Mascoma carp brain, liver, and muscle tissues, providing evidence of the presence of BMAA in the Mascoma Lake food web (Figure 1, Table 1)
Summary
It is probable that one or more environmental toxins contribute to the etiology of sporadicAmyotrophic Lateral Sclerosis (ALS), most likely interacting with underlying genetic susceptibility factors [1,2]. A neurotoxin produced by cyanobacteria, β-N-methylamino-L-alanine (BMAA) has been implicated as a potential environmental risk factor for ALS [3,4,5]. Chamorro people to make flour, might be the environmental source of interest [14,15,16,17]. BMAA, derived from cyanobacteria existing symbiotically in the coralloid roots of Cycas micronesica [18,19], was discovered in the cycad seeds [18,20,21]. Further investigation demonstrated that BMAA is mainly concentrated in proteins and was consumed by the Chamorros through multiple dietary sources, including cycad flour, flying foxes (a type of fruit bat), and other animals that fed on cycad seeds, leading to biomagnification through the food chain [5,19,22,23,24]. Accumulation of BMAA in the brains of Chamorro patients with ALS/PDC as well as brains from North American ALS, Parkinson’s disease, and Alzheimer’s disease, but not Huntington’s disease, further supported this hypothesis [3,5,19,25]
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