Spinal Muscular Atrophy autophagy profile is tissue-dependent: differential regulation between muscle and motoneurons

Spinal Muscular Atrophy autophagy profile is tissue-dependent: differential regulation between muscle and motoneurons

Abstract Spinal muscular atrophy (SMA) is a neuromuscular genetic disease caused by reduced survival motor neuron (SMN) protein. SMN is ubiquitous and deficient levels cause spinal cord motoneurons (MNs) degeneration and muscle atrophy. Nevertheless, the mechanism by which SMN reduction in muscle co...

Full description

Bibliographic Details
Main Authors: Alba Sansa, Ivan Hidalgo, Maria P. Miralles, Sandra de la Fuente, M. Jose Perez-Garcia, Francina Munell, Rosa M. Soler, Ana Garcera
Format: Article
Language:English
Published: BMC 2021-07-01
Series:Acta Neuropathologica Communications
Subjects:
Spinal muscular atrophy
Survival motor neuron
Autophagy
Neuromuscular disease
Human iPSCs
Neurodegeneration
Online Access:https://doi.org/10.1186/s40478-021-01223-5

Internet

https://doi.org/10.1186/s40478-021-01223-5

Similar Items