Induction of Survival of Motor Neuron (SMN) Protein Deficiency in Spinal Astrocytes by Small Interfering RNA as an In Vitro Model of Spinal Muscular Atrophy.


Journal

Cells
ISSN: 2073-4409
Titre abrégé: Cells
Pays: Switzerland
ID NLM: 101600052

Informations de publication

Date de publication:
05 02 2022
Historique:
received: 15 12 2021
revised: 31 01 2022
accepted: 03 02 2022
entrez: 15 2 2022
pubmed: 16 2 2022
medline: 1 4 2022
Statut: epublish

Résumé

Spinal muscular atrophy (SMA) is a motor neuron disorder leading to progressive loss of ventral horn neurons resulting in muscle wasting. Here we investigate the contribution of spinal astrocytes to the pathogenesis of late-onset SMA forms using a mouse model. Furthermore, we generated SMA-like astrocytes using survival of motor neuron (SMN) siRNA transfection techniques. In the SMA mouse model, the activation of spinal astrocytes and the reduction of the inward rectifier potassium channel K

Identifiants

pubmed: 35159369
pii: cells11030558
doi: 10.3390/cells11030558
pmc: PMC8834238
pii:
doi:

Substances chimiques

RNA, Small Interfering 0
Reactive Oxygen Species 0

Types de publication

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

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : Stiftung Universitätsmedizin Essen
ID : 20214699106

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Auteurs

Markus Leo (M)

Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, 45147 Essen, Germany.

Linda-Isabell Schmitt (LI)

Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, 45147 Essen, Germany.

Michael Fleischer (M)

Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, 45147 Essen, Germany.

Rebecca Steffen (R)

Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, 45147 Essen, Germany.

Cora Osswald (C)

Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, 45147 Essen, Germany.

Christoph Kleinschnitz (C)

Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, 45147 Essen, Germany.

Tim Hagenacker (T)

Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, 45147 Essen, Germany.

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Classifications MeSH