Abstract
There is evidence showing abnormalities in collagen from the skin of patients with sporadic Amyotrophic Lateral Sclerosis (sALS) both from Guam and elsewhere. The non-proteinogenic 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 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-proteinogenic 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. We hypothesize that the abnormalities seen in sALS collagen may result from the misincorporation of BMAA and subsequent misfolding of the collagen protein.
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