Neuropathological spectrum of Synucleinopathies
Identifieur interne :
002387 ( PascalFrancis/Corpus );
précédent :
002386;
suivant :
002388
Neuropathological spectrum of Synucleinopathies
Auteurs : Kurt A. JellingerSource :
-
Movement disorders [ 0885-3185 ] ; 2003.
RBID : Pascal:04-0080428
Descripteurs français
English descriptors
Abstract
Synucleinopathies comprise a diverse group of neurodegenerative proteinopathies that share common pathological lesions composed of aggregates of conformational and posttranslational modifications of α-synuclein in selected populations of neurons and glia. Abnormal filamentous aggregates of misfolded α-synuclein protein are the major components of Lewy bodies, dystrophic (Lewy) neurites, and the Papp-Lantos filaments in oligodendroglia and neurons in multiple system atrophy linked to degeneration of affected brain regions. The synucleinopathies include (1) Lewy body disorders and dementia with Lewy bodies, (2) multiple system atrophy (MSA), and (3) Hallervorden-Spatz disease. (I) The pathological diagnosis of Lewy body disorders and dementia with Lewy bodies is established by validated consensus criteria based on semiquantitative assessment of subcortical and cortical Lewy bodies as their common hallmarks. They are accompanied by subcortical multisystem degeneration with neuronal loss and gliosis with or without Alzheimer pathologic state. Lewy bodies also occur in numerous other disorders, including pure autonomic failure, neuroaxonal dystrophies, and various amyloidoses and tauopathies. (2) Multiple system atrophy, a sporadic, adult-onset degenerative movement disorder of unknown cause, is characterized by α-synuclein-positive glial cytoplasmic and rare neuronal inclusions throughout the central nervous system associated with striatonigral degeneration, olivopontocerebellar atrophy, and involvement of medullar and spinal autonomic nuclei. (3) In neurodegeneration with brain iron accumulation type I, or Hallervorden-Spatz disease, α-synuclein is present in axonal spheroids and glial and neuronal inclusions. While the identity of the major components of Lewy bodies suggests that a pathway leading from normal soluble to abnormal misfolded filamentous proteins is central for their pathogenesis, regardless of the primary disorder, there are conformational differences in α-synuclein between neuronal and glial aggregates, showing nonuniform mapping for its epitopes. Despite several cellular and transgenic models, it is not clear whether inclusion body formation is an adaptive/ neuroprotective or a pathogenic reaction/process generated in response to different, mostly undetermined, functional triggers linked to neurodegeneration.
Notice en format standard (ISO 2709)
Pour connaître la documentation sur le format Inist Standard.
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| A01 | 01 | 1 | | @0 0885-3185 |
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| A03 | | 1 | | @0 Mov. disord. |
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| A05 | | | | @2 18 |
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| A06 | | | | @2 6 @3 SUP |
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| A08 | 01 | 1 | ENG | @1 Neuropathological spectrum of Synucleinopathies |
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| A09 | 01 | 1 | ENG | @1 Parkinsonism and Dementia: Synucleinopathies, Tauopathies, and Beyond. Proceedings of the "Movement" Disorder Society-European Division-Sponsored Workshop, Istanbul, Turkey, 9-11 May 2002 |
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| A11 | 01 | 1 | | @1 JELLINGER (Kurt A.) |
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| A14 | 01 | | | @1 Institute of Clinical Neurobiology @2 Vienna @3 AUT @Z 1 aut. |
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| A18 | 01 | 1 | | @1 "Movement" Disorder Society. European Division @2 Milwaukee, Wisconsin @3 USA @9 patr. |
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| A20 | | | | @2 S2-S3 |
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| A21 | | | | @1 2003 |
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| A23 | 01 | | | @0 ENG |
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| A43 | 01 | | | @1 INIST @2 20953 @5 354000113058290010 |
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| A44 | | | | @0 0000 @1 © 2004 INIST-CNRS. All rights reserved. |
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| A45 | | | | @0 77 ref. |
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| A47 | 01 | 1 | | @0 04-0080428 |
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| A60 | | | | @1 P @2 C |
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| A61 | | | | @0 A |
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| A64 | 01 | 1 | | @0 Movement disorders |
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| A66 | 01 | | | @0 USA |
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| C01 | 01 | | ENG | @0 Synucleinopathies comprise a diverse group of neurodegenerative proteinopathies that share common pathological lesions composed of aggregates of conformational and posttranslational modifications of α-synuclein in selected populations of neurons and glia. Abnormal filamentous aggregates of misfolded α-synuclein protein are the major components of Lewy bodies, dystrophic (Lewy) neurites, and the Papp-Lantos filaments in oligodendroglia and neurons in multiple system atrophy linked to degeneration of affected brain regions. The synucleinopathies include (1) Lewy body disorders and dementia with Lewy bodies, (2) multiple system atrophy (MSA), and (3) Hallervorden-Spatz disease. (I) The pathological diagnosis of Lewy body disorders and dementia with Lewy bodies is established by validated consensus criteria based on semiquantitative assessment of subcortical and cortical Lewy bodies as their common hallmarks. They are accompanied by subcortical multisystem degeneration with neuronal loss and gliosis with or without Alzheimer pathologic state. Lewy bodies also occur in numerous other disorders, including pure autonomic failure, neuroaxonal dystrophies, and various amyloidoses and tauopathies. (2) Multiple system atrophy, a sporadic, adult-onset degenerative movement disorder of unknown cause, is characterized by α-synuclein-positive glial cytoplasmic and rare neuronal inclusions throughout the central nervous system associated with striatonigral degeneration, olivopontocerebellar atrophy, and involvement of medullar and spinal autonomic nuclei. (3) In neurodegeneration with brain iron accumulation type I, or Hallervorden-Spatz disease, α-synuclein is present in axonal spheroids and glial and neuronal inclusions. While the identity of the major components of Lewy bodies suggests that a pathway leading from normal soluble to abnormal misfolded filamentous proteins is central for their pathogenesis, regardless of the primary disorder, there are conformational differences in α-synuclein between neuronal and glial aggregates, showing nonuniform mapping for its epitopes. Despite several cellular and transgenic models, it is not clear whether inclusion body formation is an adaptive/ neuroprotective or a pathogenic reaction/process generated in response to different, mostly undetermined, functional triggers linked to neurodegeneration. |
|---|
| C02 | 01 | X | | @0 002B17G |
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| C03 | 01 | X | FRE | @0 Démence corps Lewy @2 NM @5 01 |
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| C03 | 01 | X | ENG | @0 Lewy body dementia @2 NM @5 01 |
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| C03 | 01 | X | SPA | @0 Demencia cuerpos Lewy @2 NM @5 01 |
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| C03 | 02 | X | FRE | @0 Atrophie multisystématisée @2 NM @5 04 |
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| C03 | 02 | X | ENG | @0 Multiple system atrophy @2 NM @5 04 |
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| C03 | 02 | X | SPA | @0 Atrofia multisistematizada @2 NM @5 04 |
|---|
| C03 | 03 | X | FRE | @0 Corps inclusion @5 07 |
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| C03 | 03 | X | ENG | @0 Inclusion body @5 07 |
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| C03 | 03 | X | SPA | @0 Cuerpo inclusión @5 07 |
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| C03 | 04 | X | FRE | @0 Anatomopathologie @5 10 |
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| C03 | 04 | X | ENG | @0 Anatomic pathology @5 10 |
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| C03 | 04 | X | SPA | @0 Anatomía patológica @5 10 |
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| C03 | 05 | X | FRE | @0 Symptomatologie @5 17 |
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| C03 | 05 | X | ENG | @0 Symptomatology @5 17 |
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| C03 | 05 | X | SPA | @0 Sintomatología @5 17 |
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| C03 | 06 | X | FRE | @0 Exploration @5 18 |
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| C03 | 06 | X | ENG | @0 Exploration @5 18 |
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| C03 | 06 | X | SPA | @0 Exploración @5 18 |
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| C03 | 07 | X | FRE | @0 Homme @5 20 |
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| C03 | 07 | X | ENG | @0 Human @5 20 |
|---|
| C03 | 07 | X | SPA | @0 Hombre @5 20 |
|---|
| C03 | 08 | X | FRE | @0 α-Synucléine @4 CD @5 96 |
|---|
| C03 | 08 | X | ENG | @0 Synuclein @4 CD @5 96 |
|---|
| C07 | 01 | X | FRE | @0 Système nerveux pathologie @5 37 |
|---|
| C07 | 01 | X | ENG | @0 Nervous system diseases @5 37 |
|---|
| C07 | 01 | X | SPA | @0 Sistema nervioso patología @5 37 |
|---|
| C07 | 02 | X | FRE | @0 Système nerveux central pathologie @5 38 |
|---|
| C07 | 02 | X | ENG | @0 Central nervous system disease @5 38 |
|---|
| C07 | 02 | X | SPA | @0 Sistema nervosio central patología @5 38 |
|---|
| C07 | 03 | X | FRE | @0 Encéphale pathologie @5 39 |
|---|
| C07 | 03 | X | ENG | @0 Cerebral disorder @5 39 |
|---|
| C07 | 03 | X | SPA | @0 Encéfalo patología @5 39 |
|---|
| C07 | 04 | X | FRE | @0 Maladie dégénérative @5 40 |
|---|
| C07 | 04 | X | ENG | @0 Degenerative disease @5 40 |
|---|
| C07 | 04 | X | SPA | @0 Enfermedad degenerativa @5 40 |
|---|
| N21 | | | | @1 054 |
|---|
| N82 | | | | @1 PSI |
|---|
|
|---|
| pR |
| A30 | 01 | 1 | ENG | @1 Parkinsonism and Dementia: Sinucleinopathies, Tauopathies, and Beyond. Workshop @3 Istanbul TUR @4 2002-05-09 |
|---|
|
|---|
Format Inist (serveur)
| NO : | PASCAL 04-0080428 INIST |
|---|
| ET : | Neuropathological spectrum of Synucleinopathies |
|---|
| AU : | JELLINGER (Kurt A.) |
|---|
| AF : | Institute of Clinical Neurobiology/Vienna/Autriche (1 aut.) |
|---|
| DT : | Publication en série; Congrès; Niveau analytique |
|---|
| SO : | Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2003; Vol. 18; No. 6 SUP; S2-S3; Bibl. 77 ref. |
|---|
| LA : | Anglais |
|---|
| EA : | Synucleinopathies comprise a diverse group of neurodegenerative proteinopathies that share common pathological lesions composed of aggregates of conformational and posttranslational modifications of α-synuclein in selected populations of neurons and glia. Abnormal filamentous aggregates of misfolded α-synuclein protein are the major components of Lewy bodies, dystrophic (Lewy) neurites, and the Papp-Lantos filaments in oligodendroglia and neurons in multiple system atrophy linked to degeneration of affected brain regions. The synucleinopathies include (1) Lewy body disorders and dementia with Lewy bodies, (2) multiple system atrophy (MSA), and (3) Hallervorden-Spatz disease. (I) The pathological diagnosis of Lewy body disorders and dementia with Lewy bodies is established by validated consensus criteria based on semiquantitative assessment of subcortical and cortical Lewy bodies as their common hallmarks. They are accompanied by subcortical multisystem degeneration with neuronal loss and gliosis with or without Alzheimer pathologic state. Lewy bodies also occur in numerous other disorders, including pure autonomic failure, neuroaxonal dystrophies, and various amyloidoses and tauopathies. (2) Multiple system atrophy, a sporadic, adult-onset degenerative movement disorder of unknown cause, is characterized by α-synuclein-positive glial cytoplasmic and rare neuronal inclusions throughout the central nervous system associated with striatonigral degeneration, olivopontocerebellar atrophy, and involvement of medullar and spinal autonomic nuclei. (3) In neurodegeneration with brain iron accumulation type I, or Hallervorden-Spatz disease, α-synuclein is present in axonal spheroids and glial and neuronal inclusions. While the identity of the major components of Lewy bodies suggests that a pathway leading from normal soluble to abnormal misfolded filamentous proteins is central for their pathogenesis, regardless of the primary disorder, there are conformational differences in α-synuclein between neuronal and glial aggregates, showing nonuniform mapping for its epitopes. Despite several cellular and transgenic models, it is not clear whether inclusion body formation is an adaptive/ neuroprotective or a pathogenic reaction/process generated in response to different, mostly undetermined, functional triggers linked to neurodegeneration. |
|---|
| CC : | 002B17G |
|---|
| FD : | Démence corps Lewy; Atrophie multisystématisée; Corps inclusion; Anatomopathologie; Symptomatologie; Exploration; Homme; α-Synucléine |
|---|
| FG : | Système nerveux pathologie; Système nerveux central pathologie; Encéphale pathologie; Maladie dégénérative |
|---|
| ED : | Lewy body dementia; Multiple system atrophy; Inclusion body; Anatomic pathology; Symptomatology; Exploration; Human; Synuclein |
|---|
| EG : | Nervous system diseases; Central nervous system disease; Cerebral disorder; Degenerative disease |
|---|
| SD : | Demencia cuerpos Lewy; Atrofia multisistematizada; Cuerpo inclusión; Anatomía patológica; Sintomatología; Exploración; Hombre |
|---|
| LO : | INIST-20953.354000113058290010 |
|---|
| ID : | 04-0080428 |
|---|
Links to Exploration step
Pascal:04-0080428
Le document en format XML
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</keywords><front><div type="abstract" xml:lang="en">Synucleinopathies comprise a diverse group of neurodegenerative proteinopathies that share common pathological lesions composed of aggregates of conformational and posttranslational modifications of α-synuclein in selected populations of neurons and glia. Abnormal filamentous aggregates of misfolded α-synuclein protein are the major components of Lewy bodies, dystrophic (Lewy) neurites, and the Papp-Lantos filaments in oligodendroglia and neurons in multiple system atrophy linked to degeneration of affected brain regions. The synucleinopathies include (1) Lewy body disorders and dementia with Lewy bodies, (2) multiple system atrophy (MSA), and (3) Hallervorden-Spatz disease. (I) The pathological diagnosis of Lewy body disorders and dementia with Lewy bodies is established by validated consensus criteria based on semiquantitative assessment of subcortical and cortical Lewy bodies as their common hallmarks. They are accompanied by subcortical multisystem degeneration with neuronal loss and gliosis with or without Alzheimer pathologic state. Lewy bodies also occur in numerous other disorders, including pure autonomic failure, neuroaxonal dystrophies, and various amyloidoses and tauopathies. (2) Multiple system atrophy, a sporadic, adult-onset degenerative movement disorder of unknown cause, is characterized by α-synuclein-positive glial cytoplasmic and rare neuronal inclusions throughout the central nervous system associated with striatonigral degeneration, olivopontocerebellar atrophy, and involvement of medullar and spinal autonomic nuclei. (3) In neurodegeneration with brain iron accumulation type I, or Hallervorden-Spatz disease, α-synuclein is present in axonal spheroids and glial and neuronal inclusions. While the identity of the major components of Lewy bodies suggests that a pathway leading from normal soluble to abnormal misfolded filamentous proteins is central for their pathogenesis, regardless of the primary disorder, there are conformational differences in α-synuclein between neuronal and glial aggregates, showing nonuniform mapping for its epitopes. Despite several cellular and transgenic models, it is not clear whether inclusion body formation is an adaptive/ neuroprotective or a pathogenic reaction/process generated in response to different, mostly undetermined, functional triggers linked to neurodegeneration.</div>
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</fA09><fA11 i1="01" i2="1"><s1>JELLINGER (Kurt A.)</s1>
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<sZ>1 aut.</sZ>
</fA14><fA18 i1="01" i2="1"><s1>"Movement" Disorder Society. European Division</s1>
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<s5>04</s5>
</fC03><fC03 i1="02" i2="X" l="SPA"><s0>Atrofia multisistematizada</s0>
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<s5>04</s5>
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<s5>20</s5>
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<s5>40</s5>
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<s5>40</s5>
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<ET>Neuropathological spectrum of Synucleinopathies</ET>
<AU>JELLINGER (Kurt A.)</AU>
<AF>Institute of Clinical Neurobiology/Vienna/Autriche (1 aut.)</AF>
<DT>Publication en série; Congrès; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2003; Vol. 18; No. 6 SUP; S2-S3; Bibl. 77 ref.</SO>
<LA>Anglais</LA>
<EA>Synucleinopathies comprise a diverse group of neurodegenerative proteinopathies that share common pathological lesions composed of aggregates of conformational and posttranslational modifications of α-synuclein in selected populations of neurons and glia. Abnormal filamentous aggregates of misfolded α-synuclein protein are the major components of Lewy bodies, dystrophic (Lewy) neurites, and the Papp-Lantos filaments in oligodendroglia and neurons in multiple system atrophy linked to degeneration of affected brain regions. The synucleinopathies include (1) Lewy body disorders and dementia with Lewy bodies, (2) multiple system atrophy (MSA), and (3) Hallervorden-Spatz disease. (I) The pathological diagnosis of Lewy body disorders and dementia with Lewy bodies is established by validated consensus criteria based on semiquantitative assessment of subcortical and cortical Lewy bodies as their common hallmarks. They are accompanied by subcortical multisystem degeneration with neuronal loss and gliosis with or without Alzheimer pathologic state. Lewy bodies also occur in numerous other disorders, including pure autonomic failure, neuroaxonal dystrophies, and various amyloidoses and tauopathies. (2) Multiple system atrophy, a sporadic, adult-onset degenerative movement disorder of unknown cause, is characterized by α-synuclein-positive glial cytoplasmic and rare neuronal inclusions throughout the central nervous system associated with striatonigral degeneration, olivopontocerebellar atrophy, and involvement of medullar and spinal autonomic nuclei. (3) In neurodegeneration with brain iron accumulation type I, or Hallervorden-Spatz disease, α-synuclein is present in axonal spheroids and glial and neuronal inclusions. While the identity of the major components of Lewy bodies suggests that a pathway leading from normal soluble to abnormal misfolded filamentous proteins is central for their pathogenesis, regardless of the primary disorder, there are conformational differences in α-synuclein between neuronal and glial aggregates, showing nonuniform mapping for its epitopes. Despite several cellular and transgenic models, it is not clear whether inclusion body formation is an adaptive/ neuroprotective or a pathogenic reaction/process generated in response to different, mostly undetermined, functional triggers linked to neurodegeneration.</EA>
<CC>002B17G</CC>
<FD>Démence corps Lewy; Atrophie multisystématisée; Corps inclusion; Anatomopathologie; Symptomatologie; Exploration; Homme; α-Synucléine</FD>
<FG>Système nerveux pathologie; Système nerveux central pathologie; Encéphale pathologie; Maladie dégénérative</FG>
<ED>Lewy body dementia; Multiple system atrophy; Inclusion body; Anatomic pathology; Symptomatology; Exploration; Human; Synuclein</ED>
<EG>Nervous system diseases; Central nervous system disease; Cerebral disorder; Degenerative disease</EG>
<SD>Demencia cuerpos Lewy; Atrofia multisistematizada; Cuerpo inclusión; Anatomía patológica; Sintomatología; Exploración; Hombre</SD>
<LO>INIST-20953.354000113058290010</LO>
<ID>04-0080428</ID>
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