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PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)
PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model
Ming Du et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)
A competition between stimulators and antagonists of Upf complex recruitment governs human nonsense-mediated mRNA decay
Guramrit Singh et al.
PLOS BIOLOGY (2008)
Posttranscriptional gene regulation by spatial rearrangement of the 3′ untranslated region
Andrea B. Eberle et al.
PLOS BIOLOGY (2008)
G418-mediated ribosomal read-through of a nonsense mutation causing autosomal recessive proximal renal tubular acidosis
Rustam Azimov et al.
AMERICAN JOURNAL OF PHYSIOLOGY-RENAL PHYSIOLOGY (2008)
Eukaryotic ribosomal RNA determinants of aminoglycoside resistance and their role in translational fidelity
Hua Fan-Minogue et al.
RNA (2008)
Negamycin binds to the wall of the nascent chain exit tunnel of the 50S ribosomal subunit
Susan J. Schroeder et al.
ANTIMICROBIAL AGENTS AND CHEMOTHERAPY (2007)
Recycling of eukaryotic posttermination ribosomal complexes
Andrey V. Pisarev et al.
CELL (2007)
A mouse model for nonsense mutation bypass therapy shows a dramatic multiday response to geneticin
Chunmei Yang et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2007)
Inhibition of nonsense-mediated mRNA decay (NMD) by a new chemical molecule reveals the dynamic of NMD factors in P-bodies
Sebastien Durand et al.
JOURNAL OF CELL BIOLOGY (2007)
PTC124 targets genetic disorders caused by nonsense mutations
Ellen M. Welch et al.
NATURE (2007)
Safety, tolerability, and pharmacokinetics of PTC124, a nonaminoglycoside nonsense mutation suppressor, following single- and multiple-dose administration to healthy male and female adult volunteers
Samit Hirawat et al.
JOURNAL OF CLINICAL PHARMACOLOGY (2007)
Nonsense-mediated mRNA decay affects nonsense transcript levels and governs response of cystic fibrosis patients to gentamicin
Liat Linde et al.
JOURNAL OF CLINICAL INVESTIGATION (2007)
The DEAD-box RNA helicase Dbp5 functions in translation termination
Thomas Gross et al.
SCIENCE (2007)
Aminoglycoside-induced ototoxicity
Erol Selimoglu
CURRENT PHARMACEUTICAL DESIGN (2007)
Aminoglycoside-induced translational read-through in disease: Overcoming nonsense mutations by pharmacogenetic therapy
L. V. Zingman et al.
CLINICAL PHARMACOLOGY & THERAPEUTICS (2007)
Redesign of aminoglycosides for treatment of human genetic diseases caused by premature stop mutations
Igor Nudelman et al.
BIOORGANIC & MEDICINAL CHEMISTRY LETTERS (2006)
Nonsense-mediated mRNA decay: target genes and functional diversification of effectors
Jan Rehwinkel et al.
TRENDS IN BIOCHEMICAL SCIENCES (2006)
Specific inhibition of nonsense-mediated mRNA decay components, SMG-1 or Upf1, rescues the phenotype of Ullrich disease fibroblasts
Fusako Usuki et al.
MOLECULAR THERAPY (2006)
Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model
Ming Du et al.
JOURNAL OF MOLECULAR MEDICINE-JMM (2006)
Aminoglycosides as potential pharmacogenetic agents in the treatment of Hailey-Hailey disease
Richard Kellermayer et al.
JOURNAL OF INVESTIGATIVE DERMATOLOGY (2006)
Aminoglycoside suppression of nonsense mutations in severe hemophilia
PD James et al.
BLOOD (2005)
Highly efficient endogenous human gene correction using designed zinc-finger nucleases
FD Urnov et al.
NATURE (2005)
A non-sequence-specific requirement for SMN protein activity: the role of aminoglycosides in inducing elevated SMN protein levels
EC Wolstencroft et al.
HUMAN MOLECULAR GENETICS (2005)
Crystal structures of complexes between aminoglycosides and decoding A site oligonucleotides: role of the number of rings and positive charges in the specific binding leading to miscoding
B Francois et al.
NUCLEIC ACIDS RESEARCH (2005)
Premature stop codons involved in muscular dystrophies show a broad spectrum of readthrough efficiencies in response to gentamicin treatment
L Bidou et al.
GENE THERAPY (2004)
Global transcriptional effects of a suppressor tRNA and the inactivation of the regulator frmR
CD Herring et al.
JOURNAL OF BACTERIOLOGY (2004)
Nonsense-mediated decay approaches the clinic
JA Holbrook et al.
NATURE GENETICS (2004)
A faux 3′-UTR promotes aberrant termination and triggers nonsense-mediated mRNA decay
N Amrani et al.
NATURE (2004)
Correction of ATM gene function by aminoglycoside-induced read-through of premature termination codons
CH Lai et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2004)
The major 5 ' determinant in stop codon read-through involves two adjacent adenines
S Tork et al.
NUCLEIC ACIDS RESEARCH (2004)
Ketolide antimicrobial activity persists after disruption of interactions with domain II of 23S rRNA
GW Novotny et al.
ANTIMICROBIAL AGENTS AND CHEMOTHERAPY (2004)
Inhibition of nonsense-mediated mRNA decay rescues the phenotype in Ullrich's disease
F Usuki et al.
ANNALS OF NEUROLOGY (2004)
Nonsense surveillance regulates expression of diverse classes of mammalian transcripts and mutes genomic noise
JT Mendell et al.
NATURE GENETICS (2004)
Negamycin restores dystrophin expression in skeletal and cardiac muscles of mdx mice
M Arakawa et al.
JOURNAL OF BIOCHEMISTRY (2003)
Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations
M Wilschanski et al.
NEW ENGLAND JOURNAL OF MEDICINE (2003)
The molecular basis for A-site mutations conferring aminoglycoside resistance:: Relationship between ribosomal susceptibility and X-ray crystal structures
P Pfister et al.
CHEMBIOCHEM (2003)
Pyranmycins, a novel class of aminoglycosides with improved acid stability: The SAR of D-pyranoses on ring III of pyranmycin
CWT Chang et al.
ORGANIC LETTERS (2002)
Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene
M Du et al.
JOURNAL OF MOLECULAR MEDICINE-JMM (2002)
Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAs in a mammalian translation system
KM Keeling et al.
JOURNAL OF MOLECULAR MEDICINE-JMM (2002)
Salicylate protects hearing and kidney function from cisplatin toxicity without compromising its oncolytic action
GM Li et al.
LABORATORY INVESTIGATION (2002)
Predominance of six different hexanucleotide recoding signals 3′ of read-through stop codons
L Harrell et al.
NUCLEIC ACIDS RESEARCH (2002)
Suppression of nonsense mutations in the dystrophin gene by a suppressor tRNA gene
AV Kiselev et al.
MOLECULAR BIOLOGY (2002)
Binding site of macrolide antibiotics on the ribosome: New resistance mutation identifies a specific interaction of ketolides with rRNA
G Garza-Ramos et al.
JOURNAL OF BACTERIOLOGY (2001)
When the message goes awry: Disease-producing mutations that influence mRNA content and performance
JT Mendell et al.
CELL (2001)
Liposome-encapsulated aminoglycosides in pre-clinical and clinical studies
R Schiffelers et al.
JOURNAL OF ANTIMICROBIAL CHEMOTHERAPY (2001)
Impact of the six nucleotides downstream of the stop codon on translation termination
O Namy et al.
EMBO REPORTS (2001)
A novel stop codon readthrough mechanism produces functional Headcase protein in Drosophila trachea
P Steneberg et al.
EMBO REPORTS (2001)
Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis
JP Clancy et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2001)
Structural origins of aminoglycoside specificity for prokaryotic ribosomes
SR Lynch et al.
JOURNAL OF MOLECULAR BIOLOGY (2001)
Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation system
M Manuvakhova et al.
RNA (2000)
In vitro analysis of the binding of ADAR2 to the pre-mRNA encoding the GluR-B R/G site
M Öhman et al.
RNA (2000)
The crystal structure of human eukaryotic release factor eRF1 - Mechanism of stop codon recognition and peptidyl-tRNA hydrolysis
HW Song et al.
CELL (2000)
Modulation of RNA function by aminoglycoside antibiotics
R Schroeder et al.
EMBO JOURNAL (2000)
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