Spliceosomal small nuclear ribonucleoprotein biogenesis defects and motor neuron selectivity in spinal muscular atrophy

Abstract The SMN protein is essential and participates in the assembly of macromolecular complexes of RNA and protein in all cells. The best-characterized function of SMN is as an assembler of spliceosomal small nuclear ribonucleoproteins (snRNPs). SMN performs this function as part of a complex wit...

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Veröffentlicht in:	Brain research 2012-06, Vol.1462, p.93-99
Hauptverfasser:	Workman, Eileen, Kolb, Stephen J, Battle, Daniel J
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals biogenesis brain Gemin genes Humans Motor Neuron Disease - genetics Motor Neuron Disease - physiopathology Motor Neurons - physiology muscular atrophy Muscular Atrophy, Spinal - genetics Muscular Atrophy, Spinal - physiopathology mutation Mutation - genetics Mutation - physiology Neurology ribonucleoproteins Ribonucleoproteins, Small Nuclear - biosynthesis Ribonucleoproteins, Small Nuclear - genetics RNA RNA-binding proteins SMA SMN snRNP Spinal muscular atrophy spliceosomes Survival motor neuron Survival of Motor Neuron 1 Protein - genetics Survival of Motor Neuron 1 Protein - physiology
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