Autologous iPSC-Derived Human Neuromuscular Junction to Model the Pathophysiology of Hereditary Spastic Paraplegia

Autologous iPSC-Derived Human Neuromuscular Junction to Model the Pathophysiology of Hereditary Spastic Paraplegia

Hereditary spastic paraplegia (HSP) is a heterogeneous group of genetic neurodegenerative disorders, characterized by progressive lower limb spasticity and weakness resulting from retrograde axonal degeneration of motor neurons (MNs). Here, we generated in vitro human neuromuscular junctions (NMJs)...

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Bibliographic Details
Main Authors: Domiziana Costamagna, Valérie Casters, Marc Beltrà, Maurilio Sampaolesi, Anja Van Campenhout, Els Ortibus, Kaat Desloovere, Robin Duelen
Format: Article
Language:English
Published: MDPI AG 2022-10-01
Series:Cells
Subjects:
hereditary spastic paraplegia
iPSC disease modeling
motor neuron differentiation
skeletal muscle differentiation
neuromuscular junction
Online Access:https://www.mdpi.com/2073-4409/11/21/3351

Internet

https://www.mdpi.com/2073-4409/11/21/3351

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