Environmental Neurotoxins Linked to a Prototypical Neurodegenerative Disease

Peter S. Spencer, C. Edwin Garner, Valerie S. Palmer, Glen E. Kisby

Research output: Chapter in Book/Report/Conference proceedingChapter

5 Scopus citations

Abstract

Western Pacific amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) is a prototypical neurodegenerative disease with a dominant or exclusive environmental etiology that is acquired early in life and expressed clinically decades later. Although the precise cause of this tauopathy is unknown, much evidence points to the traditional use of cycad seed (. Cycas spp.) for medicine and/or food, cultural practices that have declined coincident with the changing clinical face and gradual disappearance of high-incidence ALS-PDC foci on Guam, Japan (Honshu Island), and New Guinea (West Papua, Indonesia). Two neurotoxic agents in cycad seed occupy current research attention: (1) the genotoxin methylazoxymethanol (MAM, aglycone of the principal cycad toxin, cycasin), which can dramatically disrupt brain development in rodents, and (2) β-. N-methylamino-. l-alanine (. l-BMAA), a minor cycad component and globally widespread cyanobacterial product with neurotoxic properties. Solving the etiology and pathogenesis of ALS-PDC should support the identification of environmental risk factors for neuropathologically related diseases, including sporadic ALS and Alzheimer's disease.

Original languageEnglish (US)
Title of host publicationEnvironmental Factors in Neurodevelopmental and Neurodegenerative Disorders
PublisherElsevier Inc.
Pages211-252
Number of pages42
ISBN (Electronic)9780128004074
ISBN (Print)9780128002285
DOIs
StatePublished - Feb 3 2015

Keywords

  • Alzheimer's disease (AD)
  • Azoxyglycosides
  • Cycads
  • Methylazoxymethanol (MAM)
  • Western Pacific amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS-PDC)
  • β-N-methylamino-l-alanine (BMAA)

ASJC Scopus subject areas

  • Medicine(all)

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    Spencer, P. S., Garner, C. E., Palmer, V. S., & Kisby, G. E. (2015). Environmental Neurotoxins Linked to a Prototypical Neurodegenerative Disease. In Environmental Factors in Neurodevelopmental and Neurodegenerative Disorders (pp. 211-252). Elsevier Inc.. https://doi.org/10.1016/B978-0-12-800228-5.00011-X