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Enduring involvement of tau, beta-amyloid, alpha-synuclein, ubiquitin and TDP-43 pathology in the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam (ALS/PDC).
Acta Neuropathol. 2008 Dec; 116(6):625-37.AN

Abstract

Guam ALS/PDC is a severe tangle forming disorder endemic to Guam with features overlapping such neurodegenerative disorders as Alzheimer disease (AD), Parkinson disease (PD), progressive supranuclear palsy (PSP), ALS, corticobasal degeneration (CBD) and pallido-ponto-nigral degeneration (PPND). Since the prevalence is declining, we examined brain tissue from 35 clinically diagnosed Chamorro patients with ALS/PDC and two Chamorro controls autopsied between 1946 and 2006, to determine if distinct variations in the pathology could be identified up to this time. Although the age at autopsy increased by 4.5-5 years per decade, we identified no qualitative differences in pathological deposits with antibodies against tau, ubiquitin, A beta, alpha-synuclein and TDP-43, indicating that these more recently identified proteins have been involved in the neuropathogenesis over the past 6 decades. Tau and TDP-43 positive neuronal, oligodendroglial and astrocytic inclusions involving multiple nerve fiber tracts occurred in both the ALS and PDC types, reinforcing the concept that these forms are part of the same disorder. The results obtained may help to define the commonality of the Guam disease with other tangle forming disorders and may help in monitoring the epidemiological changes that are taking place.

Authors+Show Affiliations

Kinsmen Laboratory of Neurological Research, The University of British Columbia, 2255 Wesbrook Mall, Vancouver, BC, V6T 1Z3, Canada.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

18843496

Citation

Miklossy, Judith, et al. "Enduring Involvement of Tau, Beta-amyloid, Alpha-synuclein, Ubiquitin and TDP-43 Pathology in the Amyotrophic Lateral Sclerosis/parkinsonism-dementia Complex of Guam (ALS/PDC)." Acta Neuropathologica, vol. 116, no. 6, 2008, pp. 625-37.
Miklossy J, Steele JC, Yu S, et al. Enduring involvement of tau, beta-amyloid, alpha-synuclein, ubiquitin and TDP-43 pathology in the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam (ALS/PDC). Acta Neuropathol. 2008;116(6):625-37.
Miklossy, J., Steele, J. C., Yu, S., McCall, S., Sandberg, G., McGeer, E. G., & McGeer, P. L. (2008). Enduring involvement of tau, beta-amyloid, alpha-synuclein, ubiquitin and TDP-43 pathology in the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam (ALS/PDC). Acta Neuropathologica, 116(6), 625-37. https://doi.org/10.1007/s00401-008-0439-2
Miklossy J, et al. Enduring Involvement of Tau, Beta-amyloid, Alpha-synuclein, Ubiquitin and TDP-43 Pathology in the Amyotrophic Lateral Sclerosis/parkinsonism-dementia Complex of Guam (ALS/PDC). Acta Neuropathol. 2008;116(6):625-37. PubMed PMID: 18843496.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Enduring involvement of tau, beta-amyloid, alpha-synuclein, ubiquitin and TDP-43 pathology in the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam (ALS/PDC). AU - Miklossy,Judith, AU - Steele,John C, AU - Yu,Sheng, AU - McCall,Sherman, AU - Sandberg,Glenn, AU - McGeer,Edith G, AU - McGeer,Patrick L, Y1 - 2008/10/09/ PY - 2008/06/19/received PY - 2008/09/18/accepted PY - 2008/09/11/revised PY - 2008/10/10/pubmed PY - 2009/2/4/medline PY - 2008/10/10/entrez SP - 625 EP - 37 JF - Acta neuropathologica JO - Acta Neuropathol. VL - 116 IS - 6 N2 - Guam ALS/PDC is a severe tangle forming disorder endemic to Guam with features overlapping such neurodegenerative disorders as Alzheimer disease (AD), Parkinson disease (PD), progressive supranuclear palsy (PSP), ALS, corticobasal degeneration (CBD) and pallido-ponto-nigral degeneration (PPND). Since the prevalence is declining, we examined brain tissue from 35 clinically diagnosed Chamorro patients with ALS/PDC and two Chamorro controls autopsied between 1946 and 2006, to determine if distinct variations in the pathology could be identified up to this time. Although the age at autopsy increased by 4.5-5 years per decade, we identified no qualitative differences in pathological deposits with antibodies against tau, ubiquitin, A beta, alpha-synuclein and TDP-43, indicating that these more recently identified proteins have been involved in the neuropathogenesis over the past 6 decades. Tau and TDP-43 positive neuronal, oligodendroglial and astrocytic inclusions involving multiple nerve fiber tracts occurred in both the ALS and PDC types, reinforcing the concept that these forms are part of the same disorder. The results obtained may help to define the commonality of the Guam disease with other tangle forming disorders and may help in monitoring the epidemiological changes that are taking place. SN - 1432-0533 UR - https://www.unboundmedicine.com/medline/citation/18843496/Enduring_involvement_of_tau_beta_amyloid_alpha_synuclein_ubiquitin_and_TDP_43_pathology_in_the_amyotrophic_lateral_sclerosis/parkinsonism_dementia_complex_of_Guam__ALS/PDC__ L2 - https://dx.doi.org/10.1007/s00401-008-0439-2 DB - PRIME DP - Unbound Medicine ER -