Browsing Articles publicats (Medicina Experimental) by Author "Lafarga, Miguel"
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Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA
Narcís, Oriol J.; Tapia, Olga; Tarabal Mostazo, Olga; Piedrafita Llorens, Lídia; Calderó i Pardo, Jordi; Berciano, Maria T.; Lafarga, Miguel (Nature Research, 2018)Spinal muscular atrophy (SMA) is a severe motor neuron (MN) disease caused by the deletion or mutation of the survival motor neuron 1 (SMN1) gene, which results in reduced levels of the SMN protein and the selective ... -
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
Tapia, Olga; Narcís, Oriol J.; Riancho, Javier; Tarabal Mostazo, Olga; Piedrafita Llorens, Lídia; Calderó i Pardo, Jordi; Berciano, Maria T.; Lafarga, Miguel (Elsevier, 2017)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 ... -
Nusinersen ameliorates motor function and prevents motoneuron Cajal body disassembly and abnormal poly(A) RNA distribution in a SMA mouse model
Berciano, Maria T.; Puente-Bedia, Alba; Medina‑Samamé, Almundena; Rodriguez-Rey, José C.; Calderó i Pardo, Jordi; Lafarga, Miguel; Tapia, Olga (Nature Publishing Group, 2020)Spinal muscular atrophy (SMA) is a devastating autosomal recessive neuromuscular disease characterized by degeneration of spinal cord alpha motor neurons (αMNs). SMA is caused by the homozygous deletion or mutation of the ...