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Statements

Subject Item
n2:RIV%2F00216208%3A11120%2F14%3A43909188%21RIV15-MSM-11120___
rdf:type
skos:Concept n19:Vysledek
dcterms:description
Background: TDP-43 proteinopathies represent a spectrum of neurodegenerative disorders. Variable clinical presentations including frontotemporal dementia, amyotrophic lateral sclerosis (ALS) and mixed forms are associated with the spatial heterogeneity of the TDP-43 pathology. Recent studies have emphasized the role of oligodendrocytes in the pathogenesis of ALS. Objective: To evaluate whether TDP-43 proteinopathies are associated with an oligodendroglial response. Methods: We performed a study on 7 controls and 10 diseased patients with spinal cord involvement. Using the oligodendroglia-specific antibody TPPP/p25, we assessed oligodendrocyte density in the lateral corticospinal tracts (LCSs) along with the presence of perineuronal oligodendrocytes (PNOGs) in the anterior horns. We performed a densitometry of myelin basic protein (MBP) immunoreactivity. The numbers of TDP-43 and p62 immunoreactive inclusions were counted in both the LCSs and the anterior horns. Results: Double immunolabeling confirmed that oligodendrocytes harbor TDP-43 inclusions. In the LCSs, MBP density, but not the number of oligodendrocytes, was decreased in the diseased group. However, oligodendrocyte counts in the LCS correlated positively, and the density of MBP inversely, with the number of neuronal inclusions in the anterior horn, suggestive of a compensatory response of oligodendrocytes. The number of neurons with PNOGs correlated with the amount of inclusions. Conclusion: Our study further emphasizes the importance of oligodendroglia in the pathogenesis of TDP-43 proteinopathies with spinal cord involvement. (C) 2014 S. Karger AG, Basel. Background: TDP-43 proteinopathies represent a spectrum of neurodegenerative disorders. Variable clinical presentations including frontotemporal dementia, amyotrophic lateral sclerosis (ALS) and mixed forms are associated with the spatial heterogeneity of the TDP-43 pathology. Recent studies have emphasized the role of oligodendrocytes in the pathogenesis of ALS. Objective: To evaluate whether TDP-43 proteinopathies are associated with an oligodendroglial response. Methods: We performed a study on 7 controls and 10 diseased patients with spinal cord involvement. Using the oligodendroglia-specific antibody TPPP/p25, we assessed oligodendrocyte density in the lateral corticospinal tracts (LCSs) along with the presence of perineuronal oligodendrocytes (PNOGs) in the anterior horns. We performed a densitometry of myelin basic protein (MBP) immunoreactivity. The numbers of TDP-43 and p62 immunoreactive inclusions were counted in both the LCSs and the anterior horns. Results: Double immunolabeling confirmed that oligodendrocytes harbor TDP-43 inclusions. In the LCSs, MBP density, but not the number of oligodendrocytes, was decreased in the diseased group. However, oligodendrocyte counts in the LCS correlated positively, and the density of MBP inversely, with the number of neuronal inclusions in the anterior horn, suggestive of a compensatory response of oligodendrocytes. The number of neurons with PNOGs correlated with the amount of inclusions. Conclusion: Our study further emphasizes the importance of oligodendroglia in the pathogenesis of TDP-43 proteinopathies with spinal cord involvement. (C) 2014 S. Karger AG, Basel.
dcterms:title
Oligodendroglial Response in the Spinal Cord in TDP-43 Proteinopathy with Motor Neuron Involvement Oligodendroglial Response in the Spinal Cord in TDP-43 Proteinopathy with Motor Neuron Involvement
skos:prefLabel
Oligodendroglial Response in the Spinal Cord in TDP-43 Proteinopathy with Motor Neuron Involvement Oligodendroglial Response in the Spinal Cord in TDP-43 Proteinopathy with Motor Neuron Involvement
skos:notation
RIV/00216208:11120/14:43909188!RIV15-MSM-11120___
n3:aktivita
n11:I n11:S n11:Z
n3:aktivity
I, S, Z(MSM0021620849)
n3:cisloPeriodika
3
n3:dodaniDat
n14:2015
n3:domaciTvurceVysledku
n9:7678827 n9:4091507
n3:druhVysledku
n13:J
n3:duvernostUdaju
n16:S
n3:entitaPredkladatele
n5:predkladatel
n3:idSjednocenehoVysledku
34171
n3:idVysledku
RIV/00216208:11120/14:43909188
n3:jazykVysledku
n10:eng
n3:klicovaSlova
TPPP/p25; TDP-43; Spinal cord; p62; Perineuronal oligodendrocyte; Oligodendroglia; Myelin basic protein; Amyotrophic lateral sclerosis
n3:klicoveSlovo
n4:Myelin%20basic%20protein n4:Amyotrophic%20lateral%20sclerosis n4:Spinal%20cord n4:Perineuronal%20oligodendrocyte n4:p62 n4:Oligodendroglia n4:TDP-43 n4:TPPP%2Fp25
n3:kodStatuVydavatele
CH - Švýcarská konfederace
n3:kontrolniKodProRIV
[AE4B76A1E899]
n3:nazevZdroje
Neurodegenerative Diseases
n3:obor
n6:FH
n3:pocetDomacichTvurcuVysledku
2
n3:pocetTvurcuVysledku
4
n3:rokUplatneniVysledku
n14:2014
n3:svazekPeriodika
14
n3:tvurceVysledku
Rohan, Zdeněk Matěj, Radoslav
n3:wos
000344979200002
n3:zamer
n18:MSM0021620849
s:issn
1660-2854
s:numberOfPages
8
n17:doi
10.1159/000362929
n12:organizacniJednotka
11120