David M. Bedwell

Overview

Best Publications

• Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

  Marybeth Howard;Raymond A. Frizzell;Raymond A. Frizzell;David M. Bedwell

  593
  Citations

• Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation system.

  Marina Manuvakhova;Kim Keeling;David M. Bedwell

  413
  Citations

• SUPPRESSION OF A CFTR PREMATURE STOP MUTATION IN A BRONCHIAL EPITHELIAL CELL LINE

  David M. Bedwell;Anisa Kaenjak;Dale J. Benos;Zsuzsa Bebok

  395
  Citations

• The efficiency of translation termination is determined by a synergistic interplay between upstream and downstream sequences in Saccharomyces cerevisiae.

  Brian Bonetti;Lianwu Fu;James Moon;David M. Bedwell

  375
  Citations

• Therapeutics Based on Stop Codon Readthrough

  Kim M. Keeling;Xiaojiao Xue;Gwen Gunn;David M. Bedwell

  335
  Citations

• Evidence that Systemic Gentamicin Suppresses Premature Stop Mutations in Patients with Cystic Fibrosis

  J. P. Clancy;Zsuzsa Bebök;Fadel Ruiz;Chris King

  313
  Citations

• PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model

  Ming Du;Xiaoli Liu;Ellen Marie Welch;Samit Hirawat

  300
  Citations

• The spc rtbosomal protein operon of Eschenchia coli: sequence and cotranscriptlon of the rlbosomal protein genes and a protein export gene

  Douglas Pat Cerretti;Dennis Dean;Geneva R. Davis;David M. Bedwell

  279
  Citations

• GTP Hydrolysis by eRF3 Facilitates Stop Codon Decoding during Eukaryotic Translation Termination

  Joe Salas-Marco;David M. Bedwell

  232
  Citations

• Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.

  Ming Du;Julie R. Jones;Jessica Lanier;Kim M. Keeling

  218
  Citations

• Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and reduces lysosomal glycosaminoglycan accumulation.

  Kim M. Keeling;Doug A. Brooks;John J. Hopwood;Peining Li

  214
  Citations

• Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression.

  Bijoyita Roy;Westley J. Friesen;Yuki Tomizawa;John D. Leszyk

  208
  Citations

• The vacuolar Ca2+/H+ exchanger Vcx1p/Hum1p tightly controls cytosolic Ca2+ levels in S. cerevisiae

  Attila Miseta;Richard Kellermayer;David P Aiello;Lianwu Fu

  193
  Citations

• Leaky termination at premature stop codons antagonizes nonsense-mediated mRNA decay in S. cerevisiae.

  Kim M. Keeling;Jessica Lanier;Ming Du;Joe Salas-Marco

  192
  Citations

• Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats.

  Katherine L. Tuggle;Susan E. Birket;Xiaoxia Cui;Jeong Hong

  161
  Citations

• Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAs in a mammalian translation system.

  Kim M. Keeling;David M. Bedwell

  157
  Citations

• Synthetic Aminoglycosides Efficiently Suppress Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations and Are Enhanced by Ivacaftor

  Xiaojiao Xue;Venkateshwar Mutyam;Liping Tang;Silpak Biswas

  154
  Citations

• Discrimination between defects in elongation fidelity and termination efficiency provides mechanistic insights into translational readthrough.

  Joe Salas-Marco;David M. Bedwell

  138
  Citations

• Suppression of premature termination codons as a therapeutic approach

  Kim M. Keeling;Dan Wang;Sara E. Conard;David M. Bedwell

  123
  Citations

• Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases.

  Kim M. Keeling;David M. Bedwell

  122
  Citations