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Is Ross Syndrome a New Type of Synucleinopathy? A Brief Research Report
Ross syndrome (RS) is a rare peripheral autonomic system disorder characterized by tonic pupil, hyporeflexia, and segmental anhidrosis. Neuropathological studies show that RS results from the selective cholinergic nerve degeneration. However, the cause and underlying mechanisms are largely unknown....
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7361646/ https://www.ncbi.nlm.nih.gov/pubmed/32733184 http://dx.doi.org/10.3389/fnins.2020.00635 |
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author | Ma, Mingming Yao, Jing Chen, Yongkang Liu, Han Xia, Danhao Tian, Haiyan Wang, Xinxin Wu, Erxi Wang, Xuejing Ding, Xuebing |
author_facet | Ma, Mingming Yao, Jing Chen, Yongkang Liu, Han Xia, Danhao Tian, Haiyan Wang, Xinxin Wu, Erxi Wang, Xuejing Ding, Xuebing |
author_sort | Ma, Mingming |
collection | PubMed |
description | Ross syndrome (RS) is a rare peripheral autonomic system disorder characterized by tonic pupil, hyporeflexia, and segmental anhidrosis. Neuropathological studies show that RS results from the selective cholinergic nerve degeneration. However, the cause and underlying mechanisms are largely unknown. Here, we show α-synuclein accumulation in the autonomic nerve terminals in the lesser curvature of stomach of patients with RS. In addition, immunohistochemical findings demonstrate that a dominant degeneration of cholinergic fibers is exhibited in patients with RS, while main degeneration of adrenergic fibers is demonstrated in patients with pure autonomic failure in their gastrointestinal and urinary system. Our study suggests that RS belongs to α-synucleinopathies. Moreover, our findings indicate that adrenergic nerves and cholinergic nerves are not equally damaged in different types of pure autonomic dysfunctions. |
format | Online Article Text |
id | pubmed-7361646 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-73616462020-07-29 Is Ross Syndrome a New Type of Synucleinopathy? A Brief Research Report Ma, Mingming Yao, Jing Chen, Yongkang Liu, Han Xia, Danhao Tian, Haiyan Wang, Xinxin Wu, Erxi Wang, Xuejing Ding, Xuebing Front Neurosci Neuroscience Ross syndrome (RS) is a rare peripheral autonomic system disorder characterized by tonic pupil, hyporeflexia, and segmental anhidrosis. Neuropathological studies show that RS results from the selective cholinergic nerve degeneration. However, the cause and underlying mechanisms are largely unknown. Here, we show α-synuclein accumulation in the autonomic nerve terminals in the lesser curvature of stomach of patients with RS. In addition, immunohistochemical findings demonstrate that a dominant degeneration of cholinergic fibers is exhibited in patients with RS, while main degeneration of adrenergic fibers is demonstrated in patients with pure autonomic failure in their gastrointestinal and urinary system. Our study suggests that RS belongs to α-synucleinopathies. Moreover, our findings indicate that adrenergic nerves and cholinergic nerves are not equally damaged in different types of pure autonomic dysfunctions. Frontiers Media S.A. 2020-07-08 /pmc/articles/PMC7361646/ /pubmed/32733184 http://dx.doi.org/10.3389/fnins.2020.00635 Text en Copyright © 2020 Ma, Yao, Chen, Liu, Xia, Tian, Wang, Wu, Wang and Ding. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Neuroscience Ma, Mingming Yao, Jing Chen, Yongkang Liu, Han Xia, Danhao Tian, Haiyan Wang, Xinxin Wu, Erxi Wang, Xuejing Ding, Xuebing Is Ross Syndrome a New Type of Synucleinopathy? A Brief Research Report |
title | Is Ross Syndrome a New Type of Synucleinopathy? A Brief Research Report |
title_full | Is Ross Syndrome a New Type of Synucleinopathy? A Brief Research Report |
title_fullStr | Is Ross Syndrome a New Type of Synucleinopathy? A Brief Research Report |
title_full_unstemmed | Is Ross Syndrome a New Type of Synucleinopathy? A Brief Research Report |
title_short | Is Ross Syndrome a New Type of Synucleinopathy? A Brief Research Report |
title_sort | is ross syndrome a new type of synucleinopathy? a brief research report |
topic | Neuroscience |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7361646/ https://www.ncbi.nlm.nih.gov/pubmed/32733184 http://dx.doi.org/10.3389/fnins.2020.00635 |
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