Cellular bases of the RNA metabolism dysfunction in motor neurons of a murine model of spinal muscular atrophy: Role of Cajal bodies and the nucleolus

Cellular bases of the RNA metabolism dysfunction in motor neurons of a murine model of spinal muscular atrophy: Role of Cajal bodies and the nucleolus

Spinal muscular atrophy (SMA) is caused by a homozygous deletion or mutation in the survival motor neuron 1 (SMN1) gene that leads to reduced levels of SMN protein resulting in degeneration of motor neurons (MNs). The best known functions of SMN is the biogenesis of spliceosomal snRNPs. Linked to th...

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Bibliographic Details
Main Authors: Olga Tapia, Josep Oriol Narcís, Javier Riancho, Olga Tarabal, Lídia Piedrafita, Jordi Calderó, Maria T. Berciano, Miguel Lafarga
Format: Article
Language:English
Published: Elsevier 2017-12-01
Series:Neurobiology of Disease
Subjects:
Spinal muscular atrophy
Motor neurons
Cajal bodies
Nucleolar dysfunction
Protein synthesis machinery
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996117301870

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