Neurodegenerative Disease among Isolated Pacific Populations
The systematic search for etiological factors and mechanisms of pathogenesis of neurodegenerative disorders is perhaps nowhere better exemplified than in the western Pacific. During the past four decades, faculty member Ralph Garruto has led a team systematically studying hyperendemic foci—areas with remarkably high prevalences of disease—of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) in isolated Pacific Island populations. These foci occur in different cultures, in different ecological zones, and among genetically divergent populations. They have served as natural experimental models that have had a major impact on our thinking and enhanced our understanding of these and other neurodegenerative disorders, such as Alzheimer’s disease.
This project has accumulated strong cultural, epidemiological, genetic, cellular, and molecular evidence implicating environmental factors in the causation of neurodegenerative diseases. The ultimate significance of these Pacific island paradigms may well depend on our ability to comprehensively evaluate and synthesize the growing body or relevant scientific data from our long-term studies and that of related neurological disorders as well as information from widely divergent academic fields.
- Motor neuron disease-associated loss of nuclear TDP-43 is linked to DNA double-strand break repair defects
- Cerebrovascular inflammation is associated with tau pathology in Guam Parkinsonism Dementia
- A novel liquid chromatography/mass spectrometry method for determination of neurotransmitters in brain tissue: Application to human tauopathies
- TDP-43/FUS in motor neuron disease: Complexity and challenges
- Tau pathology involves protein phosphatase 2A in Parkinsonism-dementia of Guam
- Reappraisal of the nosological significance of ALS-PDC mixed cases on Guam
- C9orf72 hexanucleotide repeat expansion and Guam amyotrophic lateral sclerosis/parkinsonism dementia complex