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).
Identifieur interne : 002199 ( Main/Exploration ); précédent : 002198; suivant : 002200Enduring involvement of tau, beta-amyloid, alpha-synuclein, ubiquitin and TDP-43 pathology in the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam (ALS/PDC).
Auteurs : Judith Miklossy [Canada] ; John C. Steele ; Sheng Yu ; Sherman Mccall ; Glenn Sandberg ; Edith G. Mcgeer ; Patrick L. McgeerSource :
- Acta neuropathologica [ 1432-0533 ] ; 2008.
English descriptors
- KwdEn :
- Adult, Aged, Aged, 80 and over, Amyloid beta-Peptides (metabolism), Amyotrophic Lateral Sclerosis (metabolism), Amyotrophic Lateral Sclerosis (pathology), Autopsy, Brain (metabolism), Brain (pathology), Brain Chemistry, DNA-Binding Proteins (metabolism), Dementia (metabolism), Dementia (pathology), Female, Guam, Humans, Immunohistochemistry, Inclusion Bodies (metabolism), Inclusion Bodies (pathology), Male, Middle Aged, Neurodegenerative Diseases (metabolism), Neurodegenerative Diseases (pathology), Neurofibrillary Tangles (metabolism), Neuroglia (metabolism), Neuroglia (pathology), Neurons (metabolism), Neurons (pathology), Parkinsonian Disorders (metabolism), Parkinsonian Disorders (pathology), Ubiquitin (metabolism), alpha-Synuclein (metabolism), tau Proteins (metabolism).
- MESH :
- chemical , metabolism : Amyloid beta-Peptides, DNA-Binding Proteins, Ubiquitin, alpha-Synuclein, tau Proteins.
- geographic : Guam.
- metabolism : Amyotrophic Lateral Sclerosis, Brain, Dementia, Inclusion Bodies, Neurodegenerative Diseases, Neurofibrillary Tangles, Neuroglia, Neurons, Parkinsonian Disorders.
- pathology : Amyotrophic Lateral Sclerosis, Brain, Dementia, Inclusion Bodies, Neurodegenerative Diseases, Neuroglia, Neurons, Parkinsonian Disorders.
- Adult, Aged, Aged, 80 and over, Autopsy, Brain Chemistry, Female, Humans, Immunohistochemistry, Male, Middle Aged.
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.
DOI: 10.1007/s00401-008-0439-2
PubMed: 18843496
Affiliations:
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Steele</name></author><author><name sortKey="Yu, Sheng" sort="Yu, Sheng" uniqKey="Yu S" first="Sheng" last="Yu">Sheng Yu</name></author><author><name sortKey="Mccall, Sherman" sort="Mccall, Sherman" uniqKey="Mccall S" first="Sherman" last="Mccall">Sherman Mccall</name></author><author><name sortKey="Sandberg, Glenn" sort="Sandberg, Glenn" uniqKey="Sandberg G" first="Glenn" last="Sandberg">Glenn Sandberg</name></author><author><name sortKey="Mcgeer, Edith G" sort="Mcgeer, Edith G" uniqKey="Mcgeer E" first="Edith G" last="Mcgeer">Edith G. Mcgeer</name></author><author><name sortKey="Mcgeer, Patrick L" sort="Mcgeer, Patrick L" uniqKey="Mcgeer P" first="Patrick L" last="Mcgeer">Patrick L. 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Steele</name></author><author><name sortKey="Yu, Sheng" sort="Yu, Sheng" uniqKey="Yu S" first="Sheng" last="Yu">Sheng Yu</name></author><author><name sortKey="Mccall, Sherman" sort="Mccall, Sherman" uniqKey="Mccall S" first="Sherman" last="Mccall">Sherman Mccall</name></author><author><name sortKey="Sandberg, Glenn" sort="Sandberg, Glenn" uniqKey="Sandberg G" first="Glenn" last="Sandberg">Glenn Sandberg</name></author><author><name sortKey="Mcgeer, Edith G" sort="Mcgeer, Edith G" uniqKey="Mcgeer E" first="Edith G" last="Mcgeer">Edith G. Mcgeer</name></author><author><name sortKey="Mcgeer, Patrick L" sort="Mcgeer, Patrick L" uniqKey="Mcgeer P" first="Patrick L" last="Mcgeer">Patrick L. Mcgeer</name></author></analytic><series><title level="j">Acta neuropathologica</title><idno type="eISSN">1432-0533</idno><imprint><date when="2008" type="published">2008</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Aged</term><term>Aged, 80 and over</term><term>Amyloid beta-Peptides (metabolism)</term><term>Amyotrophic Lateral Sclerosis (metabolism)</term><term>Amyotrophic Lateral Sclerosis (pathology)</term><term>Autopsy</term><term>Brain (metabolism)</term><term>Brain (pathology)</term><term>Brain Chemistry</term><term>DNA-Binding Proteins (metabolism)</term><term>Dementia (metabolism)</term><term>Dementia (pathology)</term><term>Female</term><term>Guam</term><term>Humans</term><term>Immunohistochemistry</term><term>Inclusion Bodies (metabolism)</term><term>Inclusion Bodies (pathology)</term><term>Male</term><term>Middle Aged</term><term>Neurodegenerative Diseases (metabolism)</term><term>Neurodegenerative Diseases (pathology)</term><term>Neurofibrillary Tangles (metabolism)</term><term>Neuroglia (metabolism)</term><term>Neuroglia (pathology)</term><term>Neurons (metabolism)</term><term>Neurons (pathology)</term><term>Parkinsonian Disorders (metabolism)</term><term>Parkinsonian Disorders (pathology)</term><term>Ubiquitin (metabolism)</term><term>alpha-Synuclein (metabolism)</term><term>tau Proteins (metabolism)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Amyloid beta-Peptides</term><term>DNA-Binding Proteins</term><term>Ubiquitin</term><term>alpha-Synuclein</term><term>tau Proteins</term></keywords><keywords scheme="MESH" type="geographic" xml:lang="en"><term>Guam</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Amyotrophic Lateral Sclerosis</term><term>Brain</term><term>Dementia</term><term>Inclusion Bodies</term><term>Neurodegenerative Diseases</term><term>Neurofibrillary Tangles</term><term>Neuroglia</term><term>Neurons</term><term>Parkinsonian Disorders</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Amyotrophic Lateral Sclerosis</term><term>Brain</term><term>Dementia</term><term>Inclusion Bodies</term><term>Neurodegenerative Diseases</term><term>Neuroglia</term><term>Neurons</term><term>Parkinsonian Disorders</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>Aged, 80 and over</term><term>Autopsy</term><term>Brain Chemistry</term><term>Female</term><term>Humans</term><term>Immunohistochemistry</term><term>Male</term><term>Middle Aged</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">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.</div></front></TEI><affiliations><list><country><li>Canada</li></country></list><tree><noCountry><name sortKey="Mccall, Sherman" sort="Mccall, Sherman" uniqKey="Mccall S" first="Sherman" last="Mccall">Sherman Mccall</name><name sortKey="Mcgeer, Edith G" sort="Mcgeer, Edith G" uniqKey="Mcgeer E" first="Edith G" last="Mcgeer">Edith G. Mcgeer</name><name sortKey="Mcgeer, Patrick L" sort="Mcgeer, Patrick L" uniqKey="Mcgeer P" first="Patrick L" last="Mcgeer">Patrick L. Mcgeer</name><name sortKey="Sandberg, Glenn" sort="Sandberg, Glenn" uniqKey="Sandberg G" first="Glenn" last="Sandberg">Glenn Sandberg</name><name sortKey="Steele, John C" sort="Steele, John C" uniqKey="Steele J" first="John C" last="Steele">John C. Steele</name><name sortKey="Yu, Sheng" sort="Yu, Sheng" uniqKey="Yu S" first="Sheng" last="Yu">Sheng Yu</name></noCountry><country name="Canada"><noRegion><name sortKey="Miklossy, Judith" sort="Miklossy, Judith" uniqKey="Miklossy J" first="Judith" last="Miklossy">Judith Miklossy</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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