Abstract
The Why of Sporadic Motor Neuron Disease - Many Factors, still a Mystery?
Highlights
Motor neuron diseases comprise amyotrophic lateral sclerosis (ALS), progressive muscular atrophy, progressive bulbar palsy and pseudobulbar palsy[1]
These mutations are localized in the genes for superoxide dismutase-11 (SOD1), and in the C9orf[72] and TARDBP genes, respectively
We previously suggested a role for human endogenous retroviruses (HERVs) in melanoma and in multiple sclerosis via an altered biosynthesis of melanin of the skin and, though hypothetical, of neuromelanin, respectively[5,6]
Summary
Motor neuron diseases comprise amyotrophic lateral sclerosis (ALS), progressive muscular atrophy, progressive bulbar palsy and pseudobulbar palsy[1]. Geographic foci with an up to 100fold higher incidence of the disease have been identified in parts of Japan, Guam, and South West New Guinea and a neurotoxic non-proteogenic amino acid, beta-methylamino-L-alanine that is produced by symbiotic cyanobacteria living in the roots of Cycas micronesia has been identified to enter the diet of persons living in these foci[1] This observation clearly points to a relevance of toxins/toxicants, it seems that this is specific only for a quite small number of patients. French fries have been found to correlate inversely with ALS risk[7], whereas a very low body mass index, in particular in adolescence and young adulthood is carrying a risk to develop the disease later in life[1,2] Is this reflecting a shortage in free fatty acids in the blood as the underlying risk factor? A use of the immune protein interleukin-33, as currently investigated in other neurodegenerative diseases[17], could become an alternative means to clear such protein deposits of affected motor neurons containing SOD-1 and alpha-synuclein
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