Altered action potential waveform and shorter axonal initial segment in hiPSC-derived motor neurons with mutations in VRK1

Altered action potential waveform and shorter axonal initial segment in hiPSC-derived motor neurons with mutations in VRK1

We recently described new pathogenic variants in VRK1, in patients affected with distal Hereditary Motor Neuropathy associated with upper motor neurons signs. Specifically, we provided evidences that hiPSC-derived Motor Neurons (hiPSC-MN) from these patients display Cajal Bodies (CBs) disassembly an...

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Bibliographic Details
Main Authors: Rémi Bos, Khalil Rihan, Patrice Quintana, Lara El-Bazzal, Nathalie Bernard-Marissal, Nathalie Da Silva, Rosette Jabbour, André Mégarbané, Marc Bartoli, Frédéric Brocard, Valérie Delague
Format: Article
Language:English
Published: Elsevier 2022-03-01
Series:Neurobiology of Disease
Subjects:
Motoneuron
Action potential waveform
VRK1
hiPSC
Inherited peripheral neuropathy
Motor neuron disease
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996121003582

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http://www.sciencedirect.com/science/article/pii/S0969996121003582

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