Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study
<div><p>We aimed to characterize in vivo α-synuclein (α-syn) aggregates in skin nerves to ascertain: 1) the optimal marker to identify them; 2) possible differences between synucleinopathies that may justify the clinical variability. We studied multiple skin nerve α-syn deposits in 44 pa...
محفوظ في:
| المؤلف الرئيسي: | |
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| مؤلفون آخرون: | , , , , , , , , , , |
| منشور في: |
2018
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| الموضوعات: | |
| الوسوم: |
إضافة وسم
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| _version_ | 1864513513541599232 |
|---|---|
| author | V. Donadio (18628783) |
| author2 | A. Incensi (18628786) O. El-Agnaf (18628789) G. Rizzo (18628792) N. Vaikath (18628795) F. Del Sorbo (18628798) C. Scaglione (18628801) S. Capellari (12074707) A. Elia (7413308) M. Stanzani Maserati (18628804) R. Pantieri (18628807) R. Liguori (8202969) |
| author2_role | author author author author author author author author author author author |
| author_facet | V. Donadio (18628783) A. Incensi (18628786) O. El-Agnaf (18628789) G. Rizzo (18628792) N. Vaikath (18628795) F. Del Sorbo (18628798) C. Scaglione (18628801) S. Capellari (12074707) A. Elia (7413308) M. Stanzani Maserati (18628804) R. Pantieri (18628807) R. Liguori (8202969) |
| author_role | author |
| dc.creator.none.fl_str_mv | V. Donadio (18628783) A. Incensi (18628786) O. El-Agnaf (18628789) G. Rizzo (18628792) N. Vaikath (18628795) F. Del Sorbo (18628798) C. Scaglione (18628801) S. Capellari (12074707) A. Elia (7413308) M. Stanzani Maserati (18628804) R. Pantieri (18628807) R. Liguori (8202969) |
| dc.date.none.fl_str_mv | 2018-09-24T03:00:00Z |
| dc.identifier.none.fl_str_mv | 10.1038/s41598-018-32588-8 |
| dc.relation.none.fl_str_mv | https://figshare.com/articles/journal_contribution/Skin_-synuclein_deposits_differ_in_clinical_variants_of_synucleinopathy_an_in_vivo_study/25919467 |
| dc.rights.none.fl_str_mv | CC BY 4.0 info:eu-repo/semantics/openAccess |
| dc.subject.none.fl_str_mv | Biomedical and clinical sciences Neurosciences Synucleinopathies Skin Nerves Pure Autonomic Failure (PAF) Dementia With Lewy Bodies (DLB) Autonomic Fibers |
| dc.title.none.fl_str_mv | Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study |
| dc.type.none.fl_str_mv | Text Journal contribution info:eu-repo/semantics/publishedVersion text contribution to journal |
| description | <div><p>We aimed to characterize in vivo α-synuclein (α-syn) aggregates in skin nerves to ascertain: 1) the optimal marker to identify them; 2) possible differences between synucleinopathies that may justify the clinical variability. We studied multiple skin nerve α-syn deposits in 44 patients with synucleinopathy: 15 idiopathic Parkinson’s disease (IPD), 12 dementia with Lewy Bodies (DLB), 5 pure autonomic failure (PAF) and 12 multiple system atrophy (MSA). Ten healthy subjects were used as controls. Antibodies against native α-syn, C-terminal α-syn epitopes such as phosphorylation at serine 129 (p-syn) and to conformation-specific for α-syn mature amyloid fibrils (syn-F1) were used. We found that p-syn showed the highest sensitivity and specificity in disclosing skin α-syn deposits. In MSA abnormal deposits were only found in somatic fibers mainly at distal sites differently from PAF, IPD and DLB displaying α-syn deposits in autonomic fibers mainly at proximal sites. PAF and DLB showed the highest p-syn load with a widespread involvement of autonomic skin nerve fibers. In conclusion: 1) p-syn in skin nerves was the optimal marker for the in vivo diagnosis of synucleinopathies; 2) the localization and load differences of aggregates may help to identify specific diagnostic traits and support a different pathogenesis among synucleinopathies.</p><p> </p></div><h2>Other Information</h2> <p> Published in: Scientific Reports<br> License: <a href="https://creativecommons.org/licenses/by/4.0" target="_blank">https://creativecommons.org/licenses/by/4.0</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1038/s41598-018-32588-8" target="_blank">https://dx.doi.org/10.1038/s41598-018-32588-8</a></p> |
| eu_rights_str_mv | openAccess |
| id | Manara2_313a659a4f7d3c47573e29e47d14a2be |
| identifier_str_mv | 10.1038/s41598-018-32588-8 |
| network_acronym_str | Manara2 |
| network_name_str | Manara2 |
| oai_identifier_str | oai:figshare.com:article/25919467 |
| publishDate | 2018 |
| repository.mail.fl_str_mv | |
| repository.name.fl_str_mv | |
| repository_id_str | |
| rights_invalid_str_mv | CC BY 4.0 |
| spelling | Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo studyV. Donadio (18628783)A. Incensi (18628786)O. El-Agnaf (18628789)G. Rizzo (18628792)N. Vaikath (18628795)F. Del Sorbo (18628798)C. Scaglione (18628801)S. Capellari (12074707)A. Elia (7413308)M. Stanzani Maserati (18628804)R. Pantieri (18628807)R. Liguori (8202969)Biomedical and clinical sciencesNeurosciencesSynucleinopathiesSkin NervesPure Autonomic Failure (PAF)Dementia With Lewy Bodies (DLB)Autonomic Fibers<div><p>We aimed to characterize in vivo α-synuclein (α-syn) aggregates in skin nerves to ascertain: 1) the optimal marker to identify them; 2) possible differences between synucleinopathies that may justify the clinical variability. We studied multiple skin nerve α-syn deposits in 44 patients with synucleinopathy: 15 idiopathic Parkinson’s disease (IPD), 12 dementia with Lewy Bodies (DLB), 5 pure autonomic failure (PAF) and 12 multiple system atrophy (MSA). Ten healthy subjects were used as controls. Antibodies against native α-syn, C-terminal α-syn epitopes such as phosphorylation at serine 129 (p-syn) and to conformation-specific for α-syn mature amyloid fibrils (syn-F1) were used. We found that p-syn showed the highest sensitivity and specificity in disclosing skin α-syn deposits. In MSA abnormal deposits were only found in somatic fibers mainly at distal sites differently from PAF, IPD and DLB displaying α-syn deposits in autonomic fibers mainly at proximal sites. PAF and DLB showed the highest p-syn load with a widespread involvement of autonomic skin nerve fibers. In conclusion: 1) p-syn in skin nerves was the optimal marker for the in vivo diagnosis of synucleinopathies; 2) the localization and load differences of aggregates may help to identify specific diagnostic traits and support a different pathogenesis among synucleinopathies.</p><p> </p></div><h2>Other Information</h2> <p> Published in: Scientific Reports<br> License: <a href="https://creativecommons.org/licenses/by/4.0" target="_blank">https://creativecommons.org/licenses/by/4.0</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1038/s41598-018-32588-8" target="_blank">https://dx.doi.org/10.1038/s41598-018-32588-8</a></p>2018-09-24T03:00:00ZTextJournal contributioninfo:eu-repo/semantics/publishedVersiontextcontribution to journal10.1038/s41598-018-32588-8https://figshare.com/articles/journal_contribution/Skin_-synuclein_deposits_differ_in_clinical_variants_of_synucleinopathy_an_in_vivo_study/25919467CC BY 4.0info:eu-repo/semantics/openAccessoai:figshare.com:article/259194672018-09-24T03:00:00Z |
| spellingShingle | Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study V. Donadio (18628783) Biomedical and clinical sciences Neurosciences Synucleinopathies Skin Nerves Pure Autonomic Failure (PAF) Dementia With Lewy Bodies (DLB) Autonomic Fibers |
| status_str | publishedVersion |
| title | Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study |
| title_full | Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study |
| title_fullStr | Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study |
| title_full_unstemmed | Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study |
| title_short | Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study |
| title_sort | Skin α-synuclein deposits differ in clinical variants of synucleinopathy: an in vivo study |
| topic | Biomedical and clinical sciences Neurosciences Synucleinopathies Skin Nerves Pure Autonomic Failure (PAF) Dementia With Lewy Bodies (DLB) Autonomic Fibers |