Selected Publications

Academic Article

Year Title Altmetric
2023 Triamterene Functions as an Effective Nonsense Suppression Agent for MPS I-H (Hurler Syndrome)International Journal of Molecular Sciences.  24. 2023
2022 Ataluren suppresses a premature termination codon in an MPS I-H mouseJournal of Molecular Medicine.  100:1223-1235. 2022
2022 Ataluren Suppresses a Premature Termination Codon in an MPS I-H Mouse 2022
2021 A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletionNature Communications.  12. 2021
2020 Pharmacological approaches for targeting cystic fibrosis nonsense mutationsEuropean Journal of Medicinal Chemistry.  200. 2020
2020 A regulated NMD mouse model supports NMD inhibition as a viable therapeutic option to treat genetic diseasesDisease Models and Mechanisms.  13. 2020
2020 Mutation-Directed Therapeutics for Neurofibromatosis Type I 2020
2020 Finding sense in the context: Ribosomal profiling has shed new light on how ribosomes can ignore stop codons in messenger RNAeLife.  9. 2020
2020 A regulated NMD mouse model supports NMD inhibition as a viable therapeutic option to treat genetic diseases.Disease Models and Mechanisms2020
2017 Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequencesHuman 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 2016
2016 Discovery of clinically approved agents that promote suppression of cystic fibrosis transmembrane conductance regulator nonsense mutationsAmerican Journal of Respiratory and Critical Care Medicine.  194:1092-1103. 2016
2014 Long-term nonsense suppression therapy moderates MPS I-H disease progressionMolecular Genetics and Metabolism.  111:374-381. 2014
2014 Therapeutics based on stop codon readthroughAnnual Review of Genomics and Human Genetics.  15:371-394. 2014
2013 Attenuation of Nonsense-Mediated mRNA Decay Enhances In Vivo Nonsense SuppressionPLoS One.  8. 2013
2012 Suppression of premature termination codons as a therapeutic approachCritical 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 mouseMolecular Genetics and Metabolism.  105:116-125. 2012
2011 Suppression of nonsense mutations as a therapeutic approach to treat genetic diseasesWiley 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 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 mutationMolecular 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 modelJournal of Biological Chemistry.  284:6885-6892. 2009
2008 Distinct eRF3 Requirements Suggest Alternate eRF1 Conformations Mediate Peptide Release during Eukaryotic Translation TerminationMolecular 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 cerevisiaeClinical and Vaccine Immunology.  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 modelJournal 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 cerevisiaeMolecular 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 2005
2004 Leaky termination at premature stop codons antagonizes nonsense-mediated mRNA decay in S. cerevisiaeRNA.  10:691-703. 2004
2002 Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgeneJournal 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 systemJournal 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 accumulationHuman Molecular Genetics.  10:291-299. 2001
2000 Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation systemRNA.  6:1044-1055. 2000
1999 Diffusion-controlled crystallization apparatus for microgravity (DCAM): flight and ground-based applicationsJournal 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 microgravityJournal of Crystal Growth.  196:610-622. 1999
1994 Preliminary crystallographic studies of four crystal forms of serum albumin. 1994
1994 Three-dimensional structure of Schistosoma japonicum glutathione S-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 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


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
  • Education And Training

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

  • Kim Keeling