The neuropathology of multiple system atrophy and its therapeutic implications.
Auton Neurosci. 2018 May; 211:1-6.AN

Abstract

Multiple system atrophy (MSA) is a fatal neurodegenerative disorder characterized by the abnormal accumulation of toxic forms of the synaptic protein alpha-synuclein (α-syn) within oligodendrocytes and neurons. The presence of α-syn within oligodendrocytes in the form of glial cytoplasmic inclusions is the diagnostic hallmark of MSA. However, it has been postulated that α-syn is produced in neurons and propagates to oligodendrocytes, where unknown mechanisms lead to its accumulation. The presence of α-syn within neurons in MSA has not been so extensively studied, but it may shed light into neuropathological mechanisms leading to oligodendroglial accumulation. Here we summarize the principal neuropathological events of MSA, and discuss how a deeper knowledge of these mechanisms may help develop effective therapies targeting α-syn accumulation and spreading.

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Authors+Show Affiliations

Valera E

Department of Neurosciences, University of California, San Diego, 9500 Gilman Dr, La Jolla, CA 92093, USA. Electronic address: evaleramartin@ucsd.edu.

Masliah E

Division of Neurosciences, National Institute on Aging/NIH, 7201 Wisconsin Ave, Bethesda, MD 20814, USA. Electronic address: eliezer.masliah@nih.gov.

MeSH

AnimalsBrainDisease Models, AnimalHumansMultiple System AtrophyNeuronsNeuropathologyOligodendrogliaalpha-Synuclein

Pub Type(s)

Journal Article

Research Support, N.I.H., Extramural

Research Support, Non-U.S. Gov't

Review

Language

eng

PubMed ID

29169744

Citation

Valera, Elvira, and Eliezer Masliah. "The Neuropathology of Multiple System Atrophy and Its Therapeutic Implications." Autonomic Neuroscience : Basic & Clinical, vol. 211, 2018, pp. 1-6.

Valera E, Masliah E. The neuropathology of multiple system atrophy and its therapeutic implications. Auton Neurosci. 2018;211:1-6.

Valera, E., & Masliah, E. (2018). The neuropathology of multiple system atrophy and its therapeutic implications. Autonomic Neuroscience : Basic & Clinical, 211, 1-6. https://doi.org/10.1016/j.autneu.2017.11.002

Valera E, Masliah E. The Neuropathology of Multiple System Atrophy and Its Therapeutic Implications. Auton Neurosci. 2018;211:1-6. PubMed PMID: 29169744.

* Article titles in AMA citation format should be in sentence-case

TY - JOUR T1 - The neuropathology of multiple system atrophy and its therapeutic implications. AU - Valera,Elvira, AU - Masliah,Eliezer, Y1 - 2017/11/10/ PY - 2017/10/07/received PY - 2017/11/06/revised PY - 2017/11/08/accepted PY - 2017/11/25/pubmed PY - 2019/4/13/medline PY - 2017/11/25/entrez SP - 1 EP - 6 JF - Autonomic neuroscience : basic & clinical JO - Auton Neurosci VL - 211 N2 - Multiple system atrophy (MSA) is a fatal neurodegenerative disorder characterized by the abnormal accumulation of toxic forms of the synaptic protein alpha-synuclein (α-syn) within oligodendrocytes and neurons. The presence of α-syn within oligodendrocytes in the form of glial cytoplasmic inclusions is the diagnostic hallmark of MSA. However, it has been postulated that α-syn is produced in neurons and propagates to oligodendrocytes, where unknown mechanisms lead to its accumulation. The presence of α-syn within neurons in MSA has not been so extensively studied, but it may shed light into neuropathological mechanisms leading to oligodendroglial accumulation. Here we summarize the principal neuropathological events of MSA, and discuss how a deeper knowledge of these mechanisms may help develop effective therapies targeting α-syn accumulation and spreading. SN - 1872-7484 UR - https://www.unboundmedicine.com/medline/citation/29169744/The_neuropathology_of_multiple_system_atrophy_and_its_therapeutic_implications_ DB - PRIME DP - Unbound Medicine ER -

Tags

The neuropathology of multiple system atrophy and its therapeutic implications.Auton Neurosci. 2018 May; 211:1-6.AN