Published in J Mol Med (Berl) on January 25, 2002
PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model. Proc Natl Acad Sci U S A (2008) 2.22
Reawakening retrocyclins: ancestral human defensins active against HIV-1. PLoS Biol (2009) 1.80
Nonsense-mediated mRNA decay affects nonsense transcript levels and governs response of cystic fibrosis patients to gentamicin. J Clin Invest (2007) 1.73
Correction of ATM gene function by aminoglycoside-induced read-through of premature termination codons. Proc Natl Acad Sci U S A (2004) 1.64
Cytoplasmic and intra-nuclear binding of gentamicin does not require endocytosis. Hear Res (2005) 1.37
Ataluren as an agent for therapeutic nonsense suppression. Annu Rev Med (2012) 1.24
TRPV1 regulators mediate gentamicin penetration of cultured kidney cells. Hear Res (2005) 1.21
Rescue of non-sense mutated p53 tumor suppressor gene by aminoglycosides. Nucleic Acids Res (2010) 1.17
Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis. Curr Opin Pulm Med (2010) 1.10
Restoration of W1282X CFTR activity by enhanced expression. Am J Respir Cell Mol Biol (2007) 1.10
Nonsense-mediated decay in genetic disease: friend or foe? Mutat Res Rev Mutat Res (2014) 1.10
No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations. Am J Respir Cell Mol Biol (2007) 1.08
Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases. Wiley Interdiscip Rev RNA (2011) 1.08
In vitro and ex vivo suppression by aminoglycosides of PCDH15 nonsense mutations underlying type 1 Usher syndrome. Hum Genet (2007) 1.05
The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse. Mol Genet Metab (2011) 1.01
The effect of eukaryotic release factor depletion on translation termination in human cell lines. Nucleic Acids Res (2004) 1.01
Aminoglycosides restore full-length type VII collagen by overcoming premature termination codons: therapeutic implications for dystrophic epidermolysis bullosa. Mol Ther (2014) 0.97
New trends in aminoglycosides use. Medchemcomm (2014) 0.96
Statistical analysis of readthrough levels for nonsense mutations in mammalian cells reveals a major determinant of response to gentamicin. PLoS Genet (2012) 0.93
Mesh biocompatibility: effects of cellular inflammation and tissue remodelling. Langenbecks Arch Surg (2011) 0.93
5-azacytidine inhibits nonsense-mediated decay in a MYC-dependent fashion. EMBO Mol Med (2014) 0.91
Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation. Mol Genet Metab (2010) 0.91
Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a cystic fibrosis mouse model. J Biol Chem (2009) 0.90
Production of beta-globin and adult hemoglobin following G418 treatment of erythroid precursor cells from homozygous beta(0)39 thalassemia patients. Am J Hematol (2009) 0.90
Improved collagen type I/III ratio at the interface of gentamicin-supplemented polyvinylidenfluoride mesh materials. Langenbecks Arch Surg (2007) 0.89
Stably integrated luxCDABE for assessment of Salmonella invasion kinetics. Mol Imaging (2009) 0.88
Stop codon suppression via inhibition of eRF1 expression. RNA (2003) 0.87
Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression. Proc Natl Acad Sci U S A (2016) 0.85
Molecular dissection of translation termination mechanism identifies two new critical regions in eRF1. Nucleic Acids Res (2009) 0.85
G418-mediated ribosomal read-through of a nonsense mutation causing autosomal recessive proximal renal tubular acidosis. Am J Physiol Renal Physiol (2008) 0.80
Therapeutic suppression of premature termination codons: mechanisms and clinical considerations (review). Int J Mol Med (2014) 0.78
Readthrough strategies for therapeutic suppression of nonsense mutations in inherited metabolic disease. Mol Syndromol (2012) 0.76
Control of gene expression through the nonsense-mediated RNA decay pathway. Cell Biosci (2017) 0.75
Nonsense Suppression as an Approach to Treat Lysosomal Storage Diseases. Diseases (2016) 0.75
Antibiotics inhibit sphere-forming ability in suspension culture. Cancer Cell Int (2016) 0.75
Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene. J Mol Med (Berl) (2002) 2.34
PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model. Proc Natl Acad Sci U S A (2008) 2.22
Leaky termination at premature stop codons antagonizes nonsense-mediated mRNA decay in S. cerevisiae. RNA (2004) 2.02
GTP hydrolysis by eRF3 facilitates stop codon decoding during eukaryotic translation termination. Mol Cell Biol (2004) 1.87
Discrimination between defects in elongation fidelity and termination efficiency provides mechanistic insights into translational readthrough. J Mol Biol (2005) 1.65
Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model. J Mol Med (Berl) (2006) 1.28
Eukaryotic ribosomal RNA determinants of aminoglycoside resistance and their role in translational fidelity. RNA (2007) 1.26
Distinct eRF3 requirements suggest alternate eRF1 conformations mediate peptide release during eukaryotic translation termination. Mol Cell (2008) 1.25
Therapeutics based on stop codon readthrough. Annu Rev Genomics Hum Genet (2014) 1.23
Tpa1p is part of an mRNP complex that influences translation termination, mRNA deadenylation, and mRNA turnover in Saccharomyces cerevisiae. Mol Cell Biol (2006) 1.22
Aminoglycosides as potential pharmacogenetic agents in the treatment of Hailey-Hailey disease. J Invest Dermatol (2006) 1.22
Suppression of premature termination codons as a therapeutic approach. Crit Rev Biochem Mol Biol (2012) 1.13
Attenuation of nonsense-mediated mRNA decay enhances in vivo nonsense suppression. PLoS One (2013) 1.11
Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor. Am J Respir Cell Mol Biol (2014) 1.10
Nonsense suppression activity of PTC124 (ataluren). Proc Natl Acad Sci U S A (2009) 1.09
Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases. Wiley Interdiscip Rev RNA (2011) 1.08
Distinct paths to stop codon reassignment by the variant-code organisms Tetrahymena and Euplotes. Mol Cell Biol (2006) 1.05
Enhancement of alveolar epithelial sodium channel activity with decreased cystic fibrosis transmembrane conductance regulator expression in mouse lung. Am J Physiol Lung Cell Mol Physiol (2011) 1.02
The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse. Mol Genet Metab (2011) 1.01
Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54. J Mol Med (Berl) (2011) 1.00
Identification of eRF1 residues that play critical and complementary roles in stop codon recognition. RNA (2012) 0.96
Extracellular Ca(2+) sensing contributes to excess Ca(2+) accumulation and vacuolar fragmentation in a pmr1Delta mutant of S. cerevisiae. J Cell Sci (2003) 0.92
Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation. Mol Genet Metab (2010) 0.91
Eukaryotic release factor 1 phosphorylation by CK2 protein kinase is dynamic but has little effect on the efficiency of translation termination in Saccharomyces cerevisiae. Eukaryot Cell (2006) 0.91
Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a cystic fibrosis mouse model. J Biol Chem (2009) 0.90
Hexose phosphorylation and the putative calcium channel component Mid1p are required for the hexose-induced transient elevation of cytosolic calcium response in Saccharomyces cerevisiae. Mol Microbiol (2002) 0.88
Heterozygosity for the F508del mutation in the cystic fibrosis transmembrane conductance regulator anion channel attenuates influenza severity. J Infect Dis (2013) 0.87
Connection between stop codon reassignment and frequent use of shifty stop frameshifting. RNA (2009) 0.86
A Saccharomyces cerevisiae mutant unable to convert glucose to glucose-6-phosphate accumulates excessive glucose in the endoplasmic reticulum due to core oligosaccharide trimming. Eukaryot Cell (2003) 0.84
Inhibition of phosphoglucomutase activity by lithium alters cellular calcium homeostasis and signaling in Saccharomyces cerevisiae. Am J Physiol Cell Physiol (2005) 0.82
Intracellular glucose 1-phosphate and glucose 6-phosphate levels modulate Ca2+ homeostasis in Saccharomyces cerevisiae. J Biol Chem (2002) 0.81
The Ca2+ homeostasis defects in a pgm2Delta strain of Saccharomyces cerevisiae are caused by excessive vacuolar Ca2+ uptake mediated by the Ca2+-ATPase Pmc1p. J Biol Chem (2004) 0.79