Cargando…

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....

Descripción completa

Detalles Bibliográficos
Autores principales: Ma, Mingming, Yao, Jing, Chen, Yongkang, Liu, Han, Xia, Danhao, Tian, Haiyan, Wang, Xinxin, Wu, Erxi, Wang, Xuejing, Ding, Xuebing
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
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
_version_ 1783559385197314048
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
work_keys_str_mv AT mamingming isrosssyndromeanewtypeofsynucleinopathyabriefresearchreport
AT yaojing isrosssyndromeanewtypeofsynucleinopathyabriefresearchreport
AT chenyongkang isrosssyndromeanewtypeofsynucleinopathyabriefresearchreport
AT liuhan isrosssyndromeanewtypeofsynucleinopathyabriefresearchreport
AT xiadanhao isrosssyndromeanewtypeofsynucleinopathyabriefresearchreport
AT tianhaiyan isrosssyndromeanewtypeofsynucleinopathyabriefresearchreport
AT wangxinxin isrosssyndromeanewtypeofsynucleinopathyabriefresearchreport
AT wuerxi isrosssyndromeanewtypeofsynucleinopathyabriefresearchreport
AT wangxuejing isrosssyndromeanewtypeofsynucleinopathyabriefresearchreport
AT dingxuebing isrosssyndromeanewtypeofsynucleinopathyabriefresearchreport