Amyloid plaques in Guam amyotrophic lateral sclerosis/parkinsonism-dementia complex contain species of A beta similar to those found in the amyloid plaques of Alzheimer's disease and pathological aging.
The Guamanian amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is characterized by abundant neurofibrillary pathology and neuron loss. In contrast to Alzheimer's disease (AD), where extensive neurofibrillary lesions always occur with deposits of A beta in numerous amyloid plaques, A beta-rich amyloid plaques are absent or rare in most ALS/PDC patients. To characterize the amyloid plaques in the latter patients, we probed plaque-rich sections of their brains by immunohistochemistry using well-characterized antibodies to specific epitopes in the N and C termini of A beta as well as to defined epitopes in hyperphosphorylated tau (PHFtau). The results indicate that the species of A beta in the amyloid plaques of ALS/PDC patients resemble those detected in the amyloid plaques of cognitively intact subjects with pathological aging as well as patients with AD. However, the paucity of PHFtau-positive neurites in the ALS/PDC plaques suggests that they reflect pathological aging rather than AD.
Schmidt
M L
ML
Center for Neurodegenerative Disease Research, University of Pennsylvania, Philadelphia 19104-4283, USA.
Lee
V M
VM
Saido
T
T
Perl
D
D
Schuck
T
T
Iwatsubo
T
T
Trojanowski
J Q
JQ
D000368:Aged; D000544:Alzheimer Disease / Q000473:pathology*; D016229:Amyloid beta-Peptides / Q000032:analysis*; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D001921:Brain / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009928:Organ Specificity; D010300:Parkinson Disease / Q000473:pathology*; D058225:Plaque, Amyloid / Q000473:pathology*; D013577:Syndrome
D016229:Amyloid beta-Peptides