作者: Peter S. Spencer , C. Edwin Garner , Valerie S. Palmer , Glen E. Kisby
DOI: 10.1016/B978-0-12-800228-5.00011-X
关键词: Disease 、 Genetics 、 Neuroscience 、 Biology 、 Cycasin 、 Etiology 、 Cycas 、 Cycad 、 Amyotrophic lateral sclerosis 、 Pathogenesis 、 Tauopathy
摘要: Abstract Western Pacific amyotrophic lateral sclerosis and parkinsonism–dementia complex (ALS/PDC) is a prototypical neurodegenerative disease with dominant or exclusive environmental etiology that acquired early in life expressed clinically decades later. Although the precise cause of this tauopathy unknown, much evidence points to traditional use cycad seed (Cycas spp.) for medicine and/or food, cultural practices have declined coincident changing clinical face gradual disappearance high-incidence ALS-PDC foci on Guam, Japan (Honshu Island), New Guinea (West Papua, Indonesia). Two neurotoxic agents occupy current research attention: (1) genotoxin methylazoxymethanol (MAM, aglycone principal toxin, cycasin), which can dramatically disrupt brain development rodents, (2) β-N-methylamino- l -alanine ( -BMAA), minor component globally widespread cyanobacterial product properties. Solving pathogenesis should support identification risk factors neuropathologically related diseases, including sporadic ALS Alzheimer's disease.