The Small-Molecule Flunarizine in Spinal Muscular Atrophy Patient Fibroblasts Impacts on the Gemin Components of the SMN Complex and TDP43, an RNA-Binding Protein Relevant to Motor Neuron Diseases

The Small-Molecule Flunarizine in Spinal Muscular Atrophy Patient Fibroblasts Impacts on the Gemin Components of the SMN Complex and TDP43, an RNA-Binding Protein Relevant to Motor Neuron Diseases

The motor neurodegenerative disease spinal muscular atrophy (SMA) is caused by alterations of the survival motor neuron 1 (SMN1) gene involved in RNA metabolism. Although the disease mechanisms are not completely elucidated, SMN protein deficiency leads to abnormal small nuclear ribonucleoproteins (...

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Bibliographic Details
Main Authors: Delphine Sapaly, Perrine Delers, Jennifer Coridon, Badih Salman, Franck Letourneur, Florent Dumont, Suzie Lefebvre
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-04-01
Series:Frontiers in Molecular Biosciences
Subjects:
neurodegenerative disorders
spinal muscular atrophy
amyotrophic lateral sclerosis
RNA metabolism
nuclear bodies
SMN complex
Online Access:https://www.frontiersin.org/article/10.3389/fmolb.2020.00055/full

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