Apr 16, 2020

Glial cells involvement in spinal muscular atrophy: Could SMA be a neuroinflammatory disease?

Neurobiology of Disease
Elena AbatiStefania Corti

Abstract

Spinal muscular atrophy (SMA) is a severe, inherited disease characterized by the progressive degeneration and death of motor neurons of the anterior horns of the spinal cord, which results in muscular atrophy and weakness of variable severity. Its early-onset form is invariably fatal in early childhood, while milder forms lead to permanent disability, physical deformities and respiratory complications. Recently, two novel revolutionary therapies, antisense oligonucleotides and gene therapy, have been approved, and might prove successful in making long-term survival of these patients likely. In this perspective, a deep understanding of the pathogenic mechanisms and of their impact on the interactions between motor neurons and other cell types within the central nervous system (CNS) is crucial. Studies using SMA animal and cellular models have taught us that the survival and functionality of motor neurons is highly dependent on a whole range of other cell types, namely glial cells, which are responsible for a variety of different functions, such as neuronal trophic support, synaptic remodeling, and immune surveillance. Thus, it emerges that SMA is likely a non-cell autonomous, multifactorial disease in which the interaction of d...Continue Reading

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Mentioned in this Paper

Tissue Remodeling
Spinal Cord
Motor Neurons
Finding
Neuroglia
Multifactorial
Muscular Atrophy
Study
Immunologic Surveillance
Inflammation

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