Positions

Selected Publications

Academic Article

Year Title Altmetric
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 suppressionProceedings of the National Academy of Sciences.  113:12508-12513. 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 lungAJP - 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 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 cerevisiaeEukaryotic 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 modelThe Clinical investigator.  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 diseasesCurrent Pharmacogenomics.  3:259-269. 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 transgeneThe Clinical investigator.  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 systemThe Clinical investigator.  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.Journal of Protein Crystal Growth.  196:623-637. 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 AlbuminEuropean Journal of Biochemistry.  226:1049-1052. 1994
1994 Three-Dimensional structure of schistosoma japonicum glutathione s -transferase fused with a six-amino acid conserved neutralizing epitope of gp41 from hivProtein Science.  3:2233-2244. 1994
1994 Fusion proteins as alternate crystallization paths to difficult structure problemsProtein and Peptide Letters.  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 modelingProtein Engineering.  3:2233-2244. 1994

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
  • Education And Training

  • Bachelor of Science or Mathematics in Chemistry, University of Alabama at Birmingham 1990
  • UAB Microbiology, Postdoctoral Fellowship 2003
  • Full Name

  • Kim Keeling