Abstract
The nonproteinogenic amino acid, β-methylamino-L-alanine (BMAA;
Figure 2.7), has been postulated to be a cause of neurodegenerative diseases
that affect large numbers of people. However, at the time of publication of this
document, this hypothesis is still highly controversial and a number of inconsistencies must be clarified before its role in human disease can be assessed
with more certainty. The following section introduces and discusses these.
Interest in BMAA began as a result of a neurological disease known as
amyotrophic lateral sclerosis/Parkinsonism dementia complex (ALS/PDC)
present in the island of Guam in the Pacific (Arnold et al., 1953; Kurland
et al., 1961). ALS/PDC has also been identified in small populations in Irian
Jaya (western New Guinea) and Kii Peninsula of Japan. ALS/PDC has a spectrum of symptoms that resemble ALS, Parkinsonism and dementia. Different
types of neurological dysfunctions were commonly present in the same individual, and multiple cases were often seen within families. The disease rendered patients incapable of normal movement, produced memory decline,
cognitive deficits, and often led to premature death. In Guam, the peak incidence of the disease occurred during the 1950s and has been declining since
then (Plato et al., 2002; Plato et al., 2003). The disease seemed limited to
the indigenous population or others who had lived in Guam and adopted
local customs and diet. ALS/PDC is characterised by hyperphosphorylated
tau proteins that may assemble into masses ranging from a few molecules
to large amyloid masses that may propagate like prions (Buée et al., 2000;
Jucker & Walker, 2013). The altered proteins form neurofibrillary tangles
(NFTs), disrupting cell structure associated with loss of function and/or cell
death (Walker & LeVine, 2000; Chiti & Dobson, 2006).
Figure 2.7), has been postulated to be a cause of neurodegenerative diseases
that affect large numbers of people. However, at the time of publication of this
document, this hypothesis is still highly controversial and a number of inconsistencies must be clarified before its role in human disease can be assessed
with more certainty. The following section introduces and discusses these.
Interest in BMAA began as a result of a neurological disease known as
amyotrophic lateral sclerosis/Parkinsonism dementia complex (ALS/PDC)
present in the island of Guam in the Pacific (Arnold et al., 1953; Kurland
et al., 1961). ALS/PDC has also been identified in small populations in Irian
Jaya (western New Guinea) and Kii Peninsula of Japan. ALS/PDC has a spectrum of symptoms that resemble ALS, Parkinsonism and dementia. Different
types of neurological dysfunctions were commonly present in the same individual, and multiple cases were often seen within families. The disease rendered patients incapable of normal movement, produced memory decline,
cognitive deficits, and often led to premature death. In Guam, the peak incidence of the disease occurred during the 1950s and has been declining since
then (Plato et al., 2002; Plato et al., 2003). The disease seemed limited to
the indigenous population or others who had lived in Guam and adopted
local customs and diet. ALS/PDC is characterised by hyperphosphorylated
tau proteins that may assemble into masses ranging from a few molecules
to large amyloid masses that may propagate like prions (Buée et al., 2000;
Jucker & Walker, 2013). The altered proteins form neurofibrillary tangles
(NFTs), disrupting cell structure associated with loss of function and/or cell
death (Walker & LeVine, 2000; Chiti & Dobson, 2006).
| Original language | English |
|---|---|
| Title of host publication | Toxic cyanobacteria in water - Second edition |
| Subtitle of host publication | A guide to their public health consequences, monitoring and management |
| Editors | I. Chorus, M. Welker |
| Publisher | World Health Organization |
| Chapter | 2.7 |
| Pages | 123-136 |
| Number of pages | 13 |
| ISBN (Electronic) | 9781003081449 |
| ISBN (Print) | 9780367533311 |
| Publication status | Published - 28 Feb 2021 |
