Cellular and molecular mechanisms of neuronal degeneration: Amyotrophic lateral sclerosis, parkinsonism-demantia, and Alzheimer disease

American Journal of Human Biology : the Official Journal of the Human Biology Council
Ralph M Garruto

Abstract

Hyperendemic Pacific foci of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) represent naturally occurring models of late-onset chronic degenerative diseases that occur in different cultures, in different ecological zones, and among genetically divergent populations. These diseases occur among the Chamorros of the Mariana Islands, among the Auyu and Jakai of southern West New Guinea, and among the Japanese from the Kill Peninsula of Honshu Island. Accumulating evidence supports the hypothesis that a defect in mineral metabolism may be etiologically involved. Toxic and essential elements, such as calcium aluminum, and silicon cross the blood-brain barrier, deposit in affected neurons, and disrupt the axonal transport system, resulting in the abnormal copolymerization of cytoskeletal and amyloid β-proteins in neurons. The pathological accumulation of these and other proteins into neurofibrillary tangles (the hallmark neuropathological lesion) causes neuronal dysfunction and death. Recent immunocytochemical and biochemical studies indicate that the amyloid β-protein in Guamanian PD has an identical amino acid sequence, a similar N-terminus heterogeneity (variation in polypeptide length), and a similar immunoreac...Continue Reading

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