Intra-axonal protein aggregation in the peripheral nervous system.
Identifieur interne : 001322 ( Ncbi/Checkpoint ); précédent : 001321; suivant : 001323Intra-axonal protein aggregation in the peripheral nervous system.
Auteurs : Anne Vital [France] ; Wassillios G. Meissner ; Marie-Hélène Canron ; Marie-Laure Martin-Negrier ; Erwan Bezard ; François Tison ; Claude VitalSource :
- Journal of the peripheral nervous system : JPNS [ 1529-8027 ] ; 2014.
English descriptors
- KwdEn :
- 14-3-3 Proteins (metabolism), Aged, Aged, 80 and over, Axons (metabolism), Axons (pathology), Electromyography, Female, Humans, Male, Middle Aged, Nerve Tissue Proteins (metabolism), Parkinson Disease (pathology), Peripheral Nerves (metabolism), Peripheral Nerves (pathology), Peripheral Nervous System Diseases (pathology), alpha-Synuclein (metabolism).
- MESH :
- chemical , metabolism : 14-3-3 Proteins, Nerve Tissue Proteins, alpha-Synuclein.
- metabolism : Axons, Peripheral Nerves.
- pathology : Axons, Parkinson Disease, Peripheral Nerves, Peripheral Nervous System Diseases.
- Aged, Aged, 80 and over, Electromyography, Female, Humans, Male, Middle Aged.
Abstract
Intracellular protein aggregates are common pathological hallmarks of many neurodegenerative disorders, and a defect in axonal transport is also incriminated. Here, we studied intra-axonal abnormal protein aggregation and axonopathy by using immunohistochemistry and electron microscopy on peripheral nerve biopsies from 12 patients with chronic axonal peripheral neuropathy (PN) of unknown etiology. Among these patients, three had idiopathic Parkinson's disease (PD). Intra-axonal ubiquitin aggregates were more numerous in the patients with PD. Intra-axonal aggregates of tau AT8 were found in five patients without PD. Phosphorylated α-synuclein aggregation was absent in all cases, while intra-axonal colocalization of 14-3-3 β and ubiquitin was observed in two PD cases. Electron microscopy revealed enlarged axons crowded with organelles in six cases, including the three patients with PD, thus attesting a slowing of the axoplasmic flux. The number of ubiquitin aggregates was correlated with features of reduced axonal flux, while no such correlation was found for tau and 14-3-3 β. Age did not correlate with the number of tau, ubiquitin, and 14-3-3 aggregates. Thus, both ubiquitin and/or abnormal tau intra-axonal aggregates may be found in chronic axonal PN. Ubiquitin aggregates might reduce the axonal flux or result from a disease producing slowing of axonal transport.
DOI: 10.1111/jns5.12056
PubMed: 24494664
Affiliations:
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Meissner</name></author><author><name sortKey="Canron, Marie Helene" sort="Canron, Marie Helene" uniqKey="Canron M" first="Marie-Hélène" last="Canron">Marie-Hélène Canron</name></author><author><name sortKey="Martin Negrier, Marie Laure" sort="Martin Negrier, Marie Laure" uniqKey="Martin Negrier M" first="Marie-Laure" last="Martin-Negrier">Marie-Laure Martin-Negrier</name></author><author><name sortKey="Bezard, Erwan" sort="Bezard, Erwan" uniqKey="Bezard E" first="Erwan" last="Bezard">Erwan Bezard</name></author><author><name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="François" last="Tison">François Tison</name></author><author><name sortKey="Vital, Claude" sort="Vital, Claude" uniqKey="Vital C" first="Claude" last="Vital">Claude Vital</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2014">2014</date><idno type="RBID">pubmed:24494664</idno><idno type="pmid">24494664</idno><idno type="doi">10.1111/jns5.12056</idno><idno type="wicri:Area/PubMed/Corpus">000605</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">000605</idno><idno type="wicri:Area/PubMed/Curation">000583</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">000583</idno><idno type="wicri:Area/PubMed/Checkpoint">000583</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">000583</idno><idno type="wicri:Area/Ncbi/Merge">001322</idno><idno type="wicri:Area/Ncbi/Curation">001322</idno><idno type="wicri:Area/Ncbi/Checkpoint">001322</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Intra-axonal protein aggregation in the peripheral nervous system.</title><author><name sortKey="Vital, Anne" sort="Vital, Anne" uniqKey="Vital A" first="Anne" last="Vital">Anne Vital</name><affiliation wicri:level="3"><nlm:affiliation>Université Bordeaux; CNRS, Institut des Maladies Neurodégénératives, Bordeaux, France; Department of Pathology, Bordeaux University Hospital, Bordeaux, France.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Université Bordeaux; CNRS, Institut des Maladies Neurodégénératives, Bordeaux, France; Department of Pathology, Bordeaux University Hospital, Bordeaux</wicri:regionArea><placeName><region type="region">Nouvelle-Aquitaine</region><region type="old region">Aquitaine</region><settlement type="city">Bordeaux</settlement></placeName></affiliation></author><author><name sortKey="Meissner, Wassillios G" sort="Meissner, Wassillios G" uniqKey="Meissner W" first="Wassillios G" last="Meissner">Wassillios G. Meissner</name></author><author><name sortKey="Canron, Marie Helene" sort="Canron, Marie Helene" uniqKey="Canron M" first="Marie-Hélène" last="Canron">Marie-Hélène Canron</name></author><author><name sortKey="Martin Negrier, Marie Laure" sort="Martin Negrier, Marie Laure" uniqKey="Martin Negrier M" first="Marie-Laure" last="Martin-Negrier">Marie-Laure Martin-Negrier</name></author><author><name sortKey="Bezard, Erwan" sort="Bezard, Erwan" uniqKey="Bezard E" first="Erwan" last="Bezard">Erwan Bezard</name></author><author><name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="François" last="Tison">François Tison</name></author><author><name sortKey="Vital, Claude" sort="Vital, Claude" uniqKey="Vital C" first="Claude" last="Vital">Claude Vital</name></author></analytic><series><title level="j">Journal of the peripheral nervous system : JPNS</title><idno type="eISSN">1529-8027</idno><imprint><date when="2014" type="published">2014</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>14-3-3 Proteins (metabolism)</term><term>Aged</term><term>Aged, 80 and over</term><term>Axons (metabolism)</term><term>Axons (pathology)</term><term>Electromyography</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Nerve Tissue Proteins (metabolism)</term><term>Parkinson Disease (pathology)</term><term>Peripheral Nerves (metabolism)</term><term>Peripheral Nerves (pathology)</term><term>Peripheral Nervous System Diseases (pathology)</term><term>alpha-Synuclein (metabolism)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>14-3-3 Proteins</term><term>Nerve Tissue Proteins</term><term>alpha-Synuclein</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Axons</term><term>Peripheral Nerves</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Axons</term><term>Parkinson Disease</term><term>Peripheral Nerves</term><term>Peripheral Nervous System Diseases</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Aged</term><term>Aged, 80 and over</term><term>Electromyography</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Intracellular protein aggregates are common pathological hallmarks of many neurodegenerative disorders, and a defect in axonal transport is also incriminated. Here, we studied intra-axonal abnormal protein aggregation and axonopathy by using immunohistochemistry and electron microscopy on peripheral nerve biopsies from 12 patients with chronic axonal peripheral neuropathy (PN) of unknown etiology. Among these patients, three had idiopathic Parkinson's disease (PD). Intra-axonal ubiquitin aggregates were more numerous in the patients with PD. Intra-axonal aggregates of tau AT8 were found in five patients without PD. Phosphorylated α-synuclein aggregation was absent in all cases, while intra-axonal colocalization of 14-3-3 β and ubiquitin was observed in two PD cases. Electron microscopy revealed enlarged axons crowded with organelles in six cases, including the three patients with PD, thus attesting a slowing of the axoplasmic flux. The number of ubiquitin aggregates was correlated with features of reduced axonal flux, while no such correlation was found for tau and 14-3-3 β. Age did not correlate with the number of tau, ubiquitin, and 14-3-3 aggregates. Thus, both ubiquitin and/or abnormal tau intra-axonal aggregates may be found in chronic axonal PN. Ubiquitin aggregates might reduce the axonal flux or result from a disease producing slowing of axonal transport.</div></front></TEI><affiliations><list><country><li>France</li></country><region><li>Aquitaine</li><li>Nouvelle-Aquitaine</li></region><settlement><li>Bordeaux</li></settlement></list><tree><noCountry><name sortKey="Bezard, Erwan" sort="Bezard, Erwan" uniqKey="Bezard E" first="Erwan" last="Bezard">Erwan Bezard</name><name sortKey="Canron, Marie Helene" sort="Canron, Marie Helene" uniqKey="Canron M" first="Marie-Hélène" last="Canron">Marie-Hélène Canron</name><name sortKey="Martin Negrier, Marie Laure" sort="Martin Negrier, Marie Laure" uniqKey="Martin Negrier M" first="Marie-Laure" last="Martin-Negrier">Marie-Laure Martin-Negrier</name><name sortKey="Meissner, Wassillios G" sort="Meissner, Wassillios G" uniqKey="Meissner W" first="Wassillios G" last="Meissner">Wassillios G. Meissner</name><name sortKey="Tison, Francois" sort="Tison, Francois" uniqKey="Tison F" first="François" last="Tison">François Tison</name><name sortKey="Vital, Claude" sort="Vital, Claude" uniqKey="Vital C" first="Claude" last="Vital">Claude Vital</name></noCountry><country name="France"><region name="Nouvelle-Aquitaine"><name sortKey="Vital, Anne" sort="Vital, Anne" uniqKey="Vital A" first="Anne" last="Vital">Anne Vital</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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