Spinal Cord Pathology in Alpha-Synuclein Transgenic Mice

Accumulation of α-synuclein is observed in neurodegenerative diseases like Parkinson's disease and Multiple System Atrophy. In previous studies with transgenic C57BL/6 mice overexpressing α-synuclein carrying the mutations A53T and A30P found in Parkinson's disease or with a parkin-null ba...

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Main Authors: Sonja Mendritzki, Saskia Schmidt, Teresa Sczepan, Xin-Ran Zhu, Daniel Segelcke, Hermann Lübbert
Format: Article
Language:English
Published: Wiley 2010-01-01
Series:Parkinson's Disease
Online Access:http://dx.doi.org/10.4061/2010/375462
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author Sonja Mendritzki
Saskia Schmidt
Teresa Sczepan
Xin-Ran Zhu
Daniel Segelcke
Hermann Lübbert
author_facet Sonja Mendritzki
Saskia Schmidt
Teresa Sczepan
Xin-Ran Zhu
Daniel Segelcke
Hermann Lübbert
author_sort Sonja Mendritzki
collection DOAJ
description Accumulation of α-synuclein is observed in neurodegenerative diseases like Parkinson's disease and Multiple System Atrophy. In previous studies with transgenic C57BL/6 mice overexpressing α-synuclein carrying the mutations A53T and A30P found in Parkinson's disease or with a parkin-null background, we reported severe mitochondrial impairments in neurons and to a larger extent in glial cells of the mesencephalon. Neuron death was not observed in the brain. Here we show that the mice show severe motor impairments in behavioral tests. In addition, these mice exhibit astrocytic cell death in the spinal cord, accompanied by extensive gliosis and microglial activation. This is shown by cell death staining and immunohistochemistry. Ultrastructural analyses revealed severe mitochondrial impairments not only in astrocytes, but also in oligodendrocytes and, to a small extent, in neurons. Thus, the transgenic mice show a profound pathology in glial cells of the spinal cord.
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series Parkinson's Disease
spelling doaj-art-a81c9e5f933641ccb5ed9fde9182fe382025-02-03T06:05:30ZengWileyParkinson's Disease2042-00802010-01-01201010.4061/2010/375462375462Spinal Cord Pathology in Alpha-Synuclein Transgenic MiceSonja Mendritzki0Saskia Schmidt1Teresa Sczepan2Xin-Ran Zhu3Daniel Segelcke4Hermann Lübbert5Department of Animal Physiology, Biology, and Biotechnology, Ruhr-University Bochum, ND/5131, D-44780 Bochum, GermanyDepartment of Animal Physiology, Biology, and Biotechnology, Ruhr-University Bochum, ND/5131, D-44780 Bochum, GermanyDepartment of Animal Physiology, Biology, and Biotechnology, Ruhr-University Bochum, ND/5131, D-44780 Bochum, GermanyDepartment of Animal Physiology, Biology, and Biotechnology, Ruhr-University Bochum, ND/5131, D-44780 Bochum, GermanyDepartment of Animal Physiology, Biology, and Biotechnology, Ruhr-University Bochum, ND/5131, D-44780 Bochum, GermanyDepartment of Animal Physiology, Biology, and Biotechnology, Ruhr-University Bochum, ND/5131, D-44780 Bochum, GermanyAccumulation of α-synuclein is observed in neurodegenerative diseases like Parkinson's disease and Multiple System Atrophy. In previous studies with transgenic C57BL/6 mice overexpressing α-synuclein carrying the mutations A53T and A30P found in Parkinson's disease or with a parkin-null background, we reported severe mitochondrial impairments in neurons and to a larger extent in glial cells of the mesencephalon. Neuron death was not observed in the brain. Here we show that the mice show severe motor impairments in behavioral tests. In addition, these mice exhibit astrocytic cell death in the spinal cord, accompanied by extensive gliosis and microglial activation. This is shown by cell death staining and immunohistochemistry. Ultrastructural analyses revealed severe mitochondrial impairments not only in astrocytes, but also in oligodendrocytes and, to a small extent, in neurons. Thus, the transgenic mice show a profound pathology in glial cells of the spinal cord.http://dx.doi.org/10.4061/2010/375462
spellingShingle Sonja Mendritzki
Saskia Schmidt
Teresa Sczepan
Xin-Ran Zhu
Daniel Segelcke
Hermann Lübbert
Spinal Cord Pathology in Alpha-Synuclein Transgenic Mice
Parkinson's Disease
title Spinal Cord Pathology in Alpha-Synuclein Transgenic Mice
title_full Spinal Cord Pathology in Alpha-Synuclein Transgenic Mice
title_fullStr Spinal Cord Pathology in Alpha-Synuclein Transgenic Mice
title_full_unstemmed Spinal Cord Pathology in Alpha-Synuclein Transgenic Mice
title_short Spinal Cord Pathology in Alpha-Synuclein Transgenic Mice
title_sort spinal cord pathology in alpha synuclein transgenic mice
url http://dx.doi.org/10.4061/2010/375462
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AT xinranzhu spinalcordpathologyinalphasynucleintransgenicmice
AT danielsegelcke spinalcordpathologyinalphasynucleintransgenicmice
AT hermannlubbert spinalcordpathologyinalphasynucleintransgenicmice