Increasing Agrin Function Antagonizes Muscle Atrophy and Motor Impairment in Spinal Muscular Atrophy
Spinal muscular atrophy (SMA) is a pediatric genetic disease, characterized by motor neuron (MN) death, leading to progressive muscle weakness, respiratory failure, and, in the most severe cases, to death. Abnormalities at the neuromuscular junction (NMJ) have been reported in SMA, including neurofi...
Main Authors: | Marina Boido, Elena De Amicis, Valeria Valsecchi, Marco Trevisan, Ugo Ala, Markus A. Ruegg, Stefan Hettwer, Alessandro Vercelli |
---|---|
Format: | Article |
Language: | English |
Published: |
Frontiers Media S.A.
2018-01-01
|
Series: | Frontiers in Cellular Neuroscience |
Subjects: | |
Online Access: | http://journal.frontiersin.org/article/10.3389/fncel.2018.00017/full |
Similar Items
-
Pharmacological c-Jun NH2-Terminal Kinase (JNK) Pathway Inhibition Reduces Severity of Spinal Muscular Atrophy Disease in Mice
by: Roberta Schellino, et al.
Published: (2018-09-01) -
Neuromuscular junction pathology is correlated with differential motor unit vulnerability in spinal and bulbar muscular atrophy
by: Elana Molotsky, et al.
Published: (2022-07-01) -
Lowering EphA4 Does Not Ameliorate Disease in a Mouse Model for Severe Spinal Muscular Atrophy
by: Lindsay Poppe, et al.
Published: (2019-11-01) -
Innovating spinal muscular atrophy models in the therapeutic era
by: Ilaria Signoria, et al.
Published: (2023-09-01) -
Modeling Spinal Muscular Atrophy in Zebrafish: Current Advances and Future Perspectives
by: David Gonzalez, et al.
Published: (2024-02-01)