β-N-methylamino-l-alanine analysis in the brains of patients with Kii ALS/PDC.

Abstract

The Kii Peninsula of Japan and the island of Guam are known as high-incidence areas of amyotrophic lateral sclerosis and parkinsonism–dementia complex (ALS/PDC). The disorder is clinically characterized by a variable presentation of parkinsonism, dementia, and motor neuron symptoms and pathologically by tau protein deposits in the CNS.1 Both genetic and environmental factors are implicated in ALS/PDC pathogenesis. Exposure to β-N-methylamino-l-alanine (BMAA), a neurotoxin produced by cyanobacteria, has been proposed as a risk factor for ALS/PDC in Guam.2 It is not known whether Kii ALS/PDC shares the same etiology as Guam ALS/PDC. To address these issues, we conducted BMAA analyses of brain samples from 5 patients with Kii ALS/PDC using high-performance liquid chromatography (HPLC) and liquid chromatography–tandem mass spectrometry (LC/MS/MS). Acknowledgment: The authors thank Junko Karita for clerical assistance and Enago ( [ enago.jp ][1] ) for the English language review. The authors also thank the H.L. Castle Foundation, the W. F. Coulter Foundation, and the Margaret Q. Landenberger Foundation, as well as C. Childs, K. Farkas, and L. Atherton for research funding. [1]: http://www.enago.jp/