Published in Brain Res on August 02, 2002
NO66, a highly conserved dual location protein in the nucleolus and in a special type of synchronously replicating chromatin. Mol Biol Cell (2004) 1.21
Transcriptional profiling of differentially vulnerable motor neurons at pre-symptomatic stage in the Smn (2b/-) mouse model of spinal muscular atrophy. Acta Neuropathol Commun (2015) 0.83
Snord 3A: a molecular marker and modulator of prion disease progression. PLoS One (2013) 0.81
Monoubiquitination of survival motor neuron regulates its cellular localization and Cajal body integrity. Hum Mol Genet (2016) 0.75
Therapeutic strategies for spinal muscular atrophy: SMN and beyond. Dis Model Mech (2017) 0.75
Induced pluripotent stem cells from a spinal muscular atrophy patient. Nature (2008) 12.21
A large nucleolar U3 ribonucleoprotein required for 18S ribosomal RNA biogenesis. Nature (2002) 6.19
Pre-18S ribosomal RNA is structurally compacted into the SSU processome prior to being cleaved from nascent transcripts in Saccharomyces cerevisiae. Mol Cell (2004) 2.81
Ribosome biogenesis in the yeast Saccharomyces cerevisiae. Genetics (2013) 2.70
Ribosome biogenesis: of knobs and RNA processing. Exp Cell Res (2004) 2.70
RNA polymerase I transcription and pre-rRNA processing are linked by specific SSU processome components. Genes Dev (2004) 2.48
Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2009) 2.03
The long unwinding road of RNA helicases. Mol Cell (2007) 1.97
Combination of SMN trans-splicing and a neurotrophic factor increases the life span and body mass in a severe model of spinal muscular atrophy. Hum Gene Ther (2010) 1.93
The small-subunit processome is a ribosome assembly intermediate. Eukaryot Cell (2004) 1.84
Cardiac defects contribute to the pathology of spinal muscular atrophy models. Hum Mol Genet (2010) 1.76
When ribosomes go bad: diseases of ribosome biogenesis. Mol Biosyst (2010) 1.76
A non-sequence-specific requirement for SMN protein activity: the role of aminoglycosides in inducing elevated SMN protein levels. Hum Mol Genet (2005) 1.72
The small subunit processome in ribosome biogenesis—progress and prospects. Wiley Interdiscip Rev RNA (2011) 1.66
Identification of two novel loci for dominantly inherited familial amyotrophic lateral sclerosis. Am J Hum Genet (2003) 1.56
The sigma(70)-like motif: a eukaryotic RNA binding domain unique to a superfamily of proteins required for ribosome biogenesis. Mol Cell (2002) 1.53
Delivery of bifunctional RNAs that target an intronic repressor and increase SMN levels in an animal model of spinal muscular atrophy. Hum Mol Genet (2009) 1.53
SRp30c-dependent stimulation of survival motor neuron (SMN) exon 7 inclusion is facilitated by a direct interaction with hTra2 beta 1. Hum Mol Genet (2002) 1.53
The putative NTPase Fap7 mediates cytoplasmic 20S pre-rRNA processing through a direct interaction with Rps14. Mol Cell Biol (2005) 1.46
Components of an interdependent unit within the SSU processome regulate and mediate its activity. Mol Cell Biol (2002) 1.45
Genetics. Mysterious ribosomopathies. Science (2013) 1.44
Ribosome biogenesis is sensed at the Start cell cycle checkpoint. Mol Biol Cell (2006) 1.43
Development of a single vector system that enhances trans-splicing of SMN2 transcripts. PLoS One (2008) 1.43
The human Imp3 and Imp4 proteins form a ternary complex with hMpp10, which only interacts with the U3 snoRNA in 60-80S ribonucleoprotein complexes. Nucleic Acids Res (2003) 1.41
Restoration of SMN function: delivery of a trans-splicing RNA re-directs SMN2 pre-mRNA splicing. Mol Ther (2007) 1.39
Stimulating full-length SMN2 expression by delivering bifunctional RNAs via a viral vector. Mol Ther (2006) 1.37
Crosstalk in gene expression: coupling and co-regulation of rDNA transcription, pre-ribosome assembly and pre-rRNA processing. Curr Opin Cell Biol (2005) 1.35
Protein phosphatase 1 binds to the RNA recognition motif of several splicing factors and regulates alternative pre-mRNA processing. Hum Mol Genet (2007) 1.33
Delivery of recombinant follistatin lessens disease severity in a mouse model of spinal muscular atrophy. Hum Mol Genet (2008) 1.32
Trans-splicing-mediated improvement in a severe mouse model of spinal muscular atrophy. J Neurosci (2010) 1.32
Novel aminoglycosides increase SMN levels in spinal muscular atrophy fibroblasts. Hum Genet (2006) 1.31
The PINc domain protein Utp24, a putative nuclease, is required for the early cleavage steps in 18S rRNA maturation. Proc Natl Acad Sci U S A (2006) 1.31
A direct interaction between the Utp6 half-a-tetratricopeptide repeat domain and a specific peptide in Utp21 is essential for efficient pre-rRNA processing. Mol Cell Biol (2008) 1.30
The small subunit processome is required for cell cycle progression at G1. Mol Biol Cell (2004) 1.26
Spinal muscular atrophy: a motor neuron disorder or a multi-organ disease? J Anat (2013) 1.23
The initial U3 snoRNA:pre-rRNA base pairing interaction required for pre-18S rRNA folding revealed by in vivo chemical probing. Nucleic Acids Res (2011) 1.23
A direct interaction between the survival motor neuron protein and p53 and its relationship to spinal muscular atrophy. J Biol Chem (2001) 1.20
Delivery of a read-through inducing compound, TC007, lessens the severity of a spinal muscular atrophy animal model. Hum Mol Genet (2009) 1.19
Comprehensive mutational analysis of yeast DEXD/H box RNA helicases involved in large ribosomal subunit biogenesis. Mol Cell Biol (2006) 1.15
Effect of diet on the survival and phenotype of a mouse model for spinal muscular atrophy. Biochem Biophys Res Commun (2009) 1.14
A dimeric structure for archaeal box C/D small ribonucleoproteins. Science (2009) 1.14
A negatively acting bifunctional RNA increases survival motor neuron both in vitro and in vivo. Hum Gene Ther (2008) 1.13
The nucleolar protein Esf2 interacts directly with the DExD/H box RNA helicase, Dbp8, to stimulate ATP hydrolysis. Nucleic Acids Res (2006) 1.13
Comprehensive mutational analysis of yeast DEXD/H box RNA helicases required for small ribosomal subunit synthesis. Mol Cell Biol (2006) 1.11
The C-terminus of Utp4, mutated in childhood cirrhosis, is essential for ribosome biogenesis. Nucleic Acids Res (2010) 1.10
The COPI vesicle complex binds and moves with survival motor neuron within axons. Hum Mol Genet (2011) 1.08
Dysregulation of ubiquitin homeostasis and β-catenin signaling promote spinal muscular atrophy. J Clin Invest (2014) 1.08
A mechanism of release of calreticulin from cells during apoptosis. J Mol Biol (2010) 1.08
Minute virus of mice NS1 interacts with the SMN protein, and they colocalize in novel nuclear bodies induced by parvovirus infection. J Virol (2002) 1.08
NOL11, implicated in the pathogenesis of North American Indian childhood cirrhosis, is required for pre-rRNA transcription and processing. PLoS Genet (2012) 1.07
Bifunctional RNAs targeting the intronic splicing silencer N1 increase SMN levels and reduce disease severity in an animal model of spinal muscular atrophy. Mol Ther (2011) 1.07
Induction of full-length survival motor neuron by polyphenol botanical compounds. Hum Genet (2007) 1.06
Delayed rRNA processing results in significant ribosome biogenesis and functional defects. Mol Cell Biol (2003) 1.05
The DEAD-box RNA helicase-like Utp25 is an SSU processome component. RNA (2010) 1.04
Cruciferous vegetable consumption alters the metabolism of the dietary carcinogen 2-amino-1-methyl-6-phenylimidazo[4,5-b]pyridine (PhIP) in humans. Carcinogenesis (2004) 1.04
Delivery of therapeutic agents through intracerebroventricular (ICV) and intravenous (IV) injection in mice. J Vis Exp (2011) 1.03
Direct central nervous system delivery provides enhanced protection following vector mediated gene replacement in a severe model of spinal muscular atrophy. Biochem Biophys Res Commun (2011) 1.01
VEGF increases blood-brain barrier permeability to Evans blue dye and tetanus toxin fragment C but not adeno-associated virus in ALS mice. Brain Res (2008) 1.00
A SMNDelta7 read-through product confers functionality to the SMNDelta7 protein. Neurosci Lett (2008) 0.97
RNA-targeting approaches for neuromuscular diseases. Trends Mol Med (2009) 0.97
SMN, Gemin2 and Gemin3 associate with beta-actin mRNA in the cytoplasm of neuronal cells in vitro. J Mol Biol (2010) 0.96
SMN in spinal muscular atrophy and snRNP biogenesis. Wiley Interdiscip Rev RNA (2011) 0.93
Decreasing disease severity in symptomatic, Smn(-/-);SMN2(+/+), spinal muscular atrophy mice following scAAV9-SMN delivery. Hum Gene Ther (2012) 0.92
The Ewing's sarcoma protein interacts with the Tudor domain of the survival motor neuron protein. Brain Res Mol Brain Res (2003) 0.92
A conserved deubiquitinating enzyme controls cell growth by regulating RNA polymerase I stability. Cell Rep (2012) 0.92
The Wallerian degeneration slow (Wld(s)) gene does not attenuate disease in a mouse model of spinal muscular atrophy. Biochem Biophys Res Commun (2008) 0.92
Partial restoration of cardio-vascular defects in a rescued severe model of spinal muscular atrophy. J Mol Cell Cardiol (2012) 0.91
Monte Carlo simulation of the Spearman-Kaerber TCID50. J Clin Bioinforma (2012) 0.91
SMN-inducing compounds for the treatment of spinal muscular atrophy. Future Med Chem (2012) 0.90
Assembling a protein-protein interaction map of the SSU processome from existing datasets. PLoS One (2011) 0.90
IGF-1:tetanus toxin fragment C fusion protein improves delivery of IGF-1 to spinal cord but fails to prolong survival of ALS mice. Brain Res (2009) 0.89
The box C/D sRNP dimeric architecture is conserved across domain Archaea. RNA (2012) 0.89
Disruption of the Survival Motor Neuron (SMN) gene in pigs using ssDNA. Transgenic Res (2011) 0.87
Development and characterization of an SMN2-based intermediate mouse model of Spinal Muscular Atrophy. Hum Mol Genet (2013) 0.87
Transgenic inactivation of murine myostatin does not decrease the severity of disease in a model of Spinal Muscular Atrophy. Neuromuscul Disord (2011) 0.87
Detection of human survival motor neuron (SMN) protein in mice containing the SMN2 transgene: applicability to preclinical therapy development for spinal muscular atrophy. J Neurosci Methods (2008) 0.87
Two-hybrid Mpp10p interaction-defective Imp4 proteins are not interaction defective in vivo but do confer specific pre-rRNA processing defects in Saccharomyces cerevisiae. Nucleic Acids Res (2004) 0.86
Minute virus of mice small nonstructural protein NS2 interacts and colocalizes with the Smn protein. J Virol (2002) 0.86
Therapeutics that directly increase SMN expression to treat spinal muscular atrophy. Drug News Perspect (2010) 0.86
The spinal muscular atrophy mouse model, SMAΔ7, displays altered axonal transport without global neurofilament alterations. Acta Neuropathol (2011) 0.86
Subcutaneous administration of TC007 reduces disease severity in an animal model of SMA. BMC Neurosci (2009) 0.85
Targeting of SMN to Cajal bodies is mediated by self-association. Hum Mol Genet (2007) 0.84
A structural model for the HAT domain of Utp6 incorporating bioinformatics and genetics. Protein Eng Des Sel (2009) 0.84
Enhancement of SMN protein levels in a mouse model of spinal muscular atrophy using novel drug-like compounds. EMBO Mol Med (2013) 0.84
Identification of a tripartite import signal in the Ewing Sarcoma protein (EWS). Biochem Biophys Res Commun (2009) 0.83
Minute virus of mice small non-structural protein NS2 localizes within, but is not required for the formation of, Smn-associated autonomous parvovirus-associated replication bodies. J Gen Virol (2005) 0.83
Dissecting the role of conserved box C/D sRNA sequences in di-sRNP assembly and function. Nucleic Acids Res (2010) 0.82
Tetanus toxin fragment C fusion facilitates protein delivery to CNS neurons from cerebrospinal fluid in mice. J Neurochem (2005) 0.82
50bp deletion in the promoter for superoxide dismutase 1 (SOD1) reduces SOD1 expression in vitro and may correlate with increased age of onset of sporadic amyotrophic lateral sclerosis. Amyotroph Lateral Scler (2008) 0.81
Probing the yeast proteome for RNA-processing factors. Genome Biol (2003) 0.81
Metabolism of nicotine and induction of CYP1A forms in precision-cut rat liver and lung slices. Toxicol In Vitro (2004) 0.81
Optimization of SMN trans-splicing through the analysis of SMN introns. J Mol Neurosci (2011) 0.81
SMN and the Gemin proteins form sub-complexes that localise to both stationary and dynamic neurite granules. Biochem Biophys Res Commun (2010) 0.80
Replacement of huntingtin exon 1 by trans-splicing. Cell Mol Life Sci (2012) 0.80
A glial cell line-derived neurotrophic factor (GDNF):tetanus toxin fragment C protein conjugate improves delivery of GDNF to spinal cord motor neurons in mice. Brain Res (2006) 0.80
Mo2 paddlewheel complexes functionalized with a single MLCT, S1 infrared-active carboxylate reporter ligand: preparation and studies of ground and photoexcited states. Inorg Chem (2013) 0.80