Positions

Selected Publications

Academic Article

Year Title Altmetric
2022 Ataluren suppresses a premature termination codon in an MPS I-H mouse.  Journal of Molecular Medicine.  100:1223-1235. 2022
2021 A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion.  Nature Communications.  12. 2021
2020 Pharmacological approaches for targeting cystic fibrosis nonsense mutations.  European Journal of Medicinal Chemistry.  200. 2020
2020 A regulated NMD mouse model supports NMD inhibition as a viable therapeutic option to treat genetic diseases.  Disease Models and Mechanisms.  13. 2020
2020 Mutation-Directed Therapeutics for Neurofibromatosis Type I.  Molecular Therapy : Nucleic Acids.  20:739-753. 2020
2020 Finding sense in the context: Ribosomal profiling has shed new light on how ribosomes can ignore stop codons in messenger RNA.  eLife.  9. 2020
2020 A regulated NMD mouse model supports NMD inhibition as a viable therapeutic option to treat genetic diseases..  Disease Models and Mechanisms.  2020
2017 Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences.  Human Molecular Genetics.  26:3116-3129. 2017
2016 Nonsense Suppression as an Approach to Treat Lysosomal Storage Diseases..  Diseases.  4. 2016
2016 Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression.  Proceedings of the National Academy of Sciences of USA.  113:12508-12513. 2016
2016 Discovery of clinically approved agents that promote suppression of cystic fibrosis transmembrane conductance regulator nonsense mutations.  American Journal of Respiratory and Critical Care Medicine.  194:1092-1103. 2016
2014 Long-term nonsense suppression therapy moderates MPS I-H disease progression.  Molecular Genetics and Metabolism.  111:374-381. 2014
2014 Therapeutics based on stop codon readthrough.  Annual Review of Genomics and Human Genetics.  15:371-394. 2014
2013 Attenuation of Nonsense-Mediated mRNA Decay Enhances In Vivo Nonsense Suppression.  PLoS One.  8. 2013
2012 Suppression of premature termination codons as a therapeutic approach.  Critical Reviews in Biochemistry and Molecular Biology.  47:444-463. 2012
2012 The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse.  Molecular Genetics and Metabolism.  105:116-125. 2012
2011 Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases.  Wiley Interdisciplinary Reviews: RNA.  2:837-852. 2011
2011 Enhancement of alveolar epithelial sodium channel activity with decreased cystic fibrosis transmembrane conductance regulator expression in mouse lung.  American Journal of Physiology: Lung Cellular and Molecular Physiology.  301. 2011
2010 Corrigendum to "Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation" [Mol. Genet. Metab. 99 (2010) 62-71] (DOI:10.1016/j.ymgme.2009.08.002).  Molecular Genetics and Metabolism.  99:439. 2010
2010 Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.  Molecular Genetics and Metabolism.  99:62-71. 2010
2009 Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a cystic fibrosis Mouse model.  Journal of Biological Chemistry.  284:6885-6892. 2009
2008 Distinct eRF3 Requirements Suggest Alternate eRF1 Conformations Mediate Peptide Release during Eukaryotic Translation Termination.  Molecular Cell.  30:599-609. 2008
2006 Eukaryotic release factor 1 phosphorylation by CK2 protein kinase is dynamic but has little effect on the efficiency of translation termination in Saccharomyces cerevisiae.  Eukaryotic Cell.  5:1378-1387. 2006
2006 Clinical doses of amikacin provide more effective suppression of the human CFTR-G542X stop mutation than gentamicin in a transgenic CF mouse model.  Journal of Molecular Medicine.  84:573-582. 2006
2006 Tpa1p is part of an mRNP complex that influences translation termination, mRNA deadenylation, and mRNA turnover in Saccharomyces cerevisiae.  Molecular and Cellular Biology.  26:5237-5248. 2006
2006 Aminoglycosides as potential pharmacogenetic agents in the treatment of Hailey-Hailey disease [2].  Journal of Investigative Dermatology.  126:229-231. 2006
2005 Pharmacological suppression of premature stop mutations that cause genetic diseases.  Current Pharmacogenomics and Personalized Medicine: the international journal for expert reviews in pharmacogenomics.  3:259-269. 2005
2004 Leaky termination at premature stop codons antagonizes nonsense-mediated mRNA decay in S. cerevisiae.  RNA.  10:691-703. 2004
2002 Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene.  Journal of Molecular Medicine.  80:595-604. 2002
2002 Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAS in a mammalian translation system.  Journal of Molecular Medicine.  80:367-376. 2002
2001 Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of α-L-iduronidase activity and reduces lysosomal glycosaminoglycan accumulation.  Human Molecular Genetics.  10:291-299. 2001
2000 Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation system.  RNA.  6:1044-1055. 2000
1999 Diffusion-controlled crystallization apparatus for microgravity (DCAM): flight and ground-based applications.  Journal of Crystal Growth.  196:602-609. 1999
1999 Lower dimer impurity incorporation may result in higher perfection of HEWL crystals grown in microgravity, a case study. 1999
1999 PCAM: a multi-user facility-based protein crystallization apparatus for microgravity.  Journal of Crystal Growth.  196:610-622. 1999
1994 Preliminary Crystallographic Studies of Four Crystal forms of Serum Albumin.  The Federation of European Biochemical Societies (FEBS) Journal.  226:1049-1052. 1994
1994 Three-Dimensional structure ofschistosoma japonicumglutathiones-transferase fused with a six-amino acid conserved neutralizing epitope of gp41 from hiv.  Protein Science.  3:2233-2244. 1994
1994 Fusion proteins as alternate crystallization paths to difficult structure problems.  Protein and Peptide Letters: international journal for rapid publication of short papers in protein and peptide science.  1:175-178. 1994
1994 Interactions between an Fab fragment against gp41 of HIV-1 and its peptide epitope: characterization using a peptide epitope library and molecular modeling 1994
Ataluren Suppresses a Premature Termination Codon in an MPS I-H Mouse

Chapter

Year Title Altmetric
2010 Recoding Therapies for Genetic Diseases 2010
2005 Therapies of Nonsense-Associated Diseases.  121-136. 2005

Research Overview

  • translation
    translation termination
    mRNA stability
    mRNA turnover
    rare or orphan diseases
    mucopolysaccharidosis I-Hurler (MPS I-H)
    lysosomal storage disease
    cystic fibrosis

    • Principal Investigator On

    Investigator On

    Education And Training

  • UAB Microbiology, Postdoctoral Fellowship
  • Bachelor of Science or Mathematics in Chemistry, University of Alabama at Birmingham 1990

    • Full Name

  • Kim Keeling