The loss of the snoRNP chaperone Nopp140 from Cajal bodies of patient fibroblasts correlates with the severity of spinal muscular atrophy

Is Good Housekeeping the Key to Motor Neuron Survival?

(2008) Kevin Talbot et al.   CELL

SMN Deficiency Causes Tissue-Specific Perturbations in the Repertoire of snRNAs and Widespread Defects in Splicing

(2008) Zhenxi Zhang et al.   CELL

Deciphering the assembly pathway of Sm-class U snRNPs

(2008) Nils Neuenkirchen et al.   FEBS LETTERS

Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle-specific SMN expression has no phenotypic effect

(2008) T. O. Gavrilina et al.   HUMAN MOLECULAR GENETICS

Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy

(2008) Shingo Kariya et al.   HUMAN MOLECULAR GENETICS

The Hsp90 chaperone controls the biogenesis of L7Ae RNPs through conserved machinery

(2008) Séverine Boulon et al.   JOURNAL OF CELL BIOLOGY