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Kim Keeling
Assistant Professor
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kkeeling@uab.edu
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Kim Keeling
Assistant Professor
Positions
Associate Scientist (C)
,
Cystic Fibrosis Research Center
,
School of Medicine
2011 -
Associate Scientist (C)
,
Comprehensive Arthritis, Musculoskeletal, Bone and Autoimmunity Center (CAMBAC)
,
School of Medicine
2015 -
Assistant Professor (P)
,
Biochemistry & Molecular Genetics
,
Academic Joint Departments
2016 -
Publications
Research
Background
Contact
Publications
Selected Publications
Academic Article
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Year
Title
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2020
Pharmacological approaches for targeting cystic fibrosis nonsense mutations
2020
2020
A regulated NMD mouse model supports NMD inhibition as a viable therapeutic option to treat genetic diseases
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
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
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
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
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
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
2002
2002
Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAS in a mammalian translation system
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.
.
Journal of Protein Crystal Growth
. 196:623-637.
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
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
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
.
Protein Engineering
. 3:2233-2244.
1994
Chapter
Filter
Year
Title
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2010
Recoding Therapies for Genetic Diseases
2010
2005
Therapies of Nonsense-Associated Diseases
. 121-136.
2005
Research
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
Investigating Nonsense-Mediated mRNA Decay as a Therapeutic Target
awarded by
Cystic Fibrosis Foundation
2020 - 2022
Investigating the Effects of Reducing Nonsense-Mediated mRNA Decay Efficiency
awarded by
NIH - OFFICE OF THE DIRECTOR
2016 - 2019
Private Grant
awarded by
ELOXX PHARMACEUTICALS
2016 - 2018
Private Grant
awarded by
ELOXX PHARMACEUTICALS
2016 - 2018
Increasing CFTR Expression by NMD Attenuation
awarded by
Cystic Fibrosis Foundation
2016 - 2017
Private Grant
awarded by
ELOXX PHARMACEUTICALS
2015 - 2017
Private Grant
awarded by
Novartis Pharma AG
2014 - 2016
Private Grant
awarded by
NOVARTIS PHARMACEUTICALS CORPORATION
2013 - 2015
Investigator On
Host Factors in Response to Therapeutic Monoclonal Antibodies and Vaccination
awarded by
National Institute of Allergy and Infectious Diseases/NIH/DHHS
2020 - 2025
Exploring Nonsense Suppression as a Treatment for NF1
awarded by
GILBERT FAMILY FOUNDATION'S GENE THERAPY INITIATIVE, LLC (GTI)
2018 - 2021
Amino Acids Incorporated at CFTR Nonsense Mutations During Readthrough
awarded by
Cystic Fibrosis Foundation
2019 - 2021
Private Grant
awarded by
MONTE ROSA THERAPEUTICS AG
2020 - 2021
Private Grant
awarded by
ZIKANI THERAPEUTICS, INC
2019 - 2020
Identify Drugs to Treat MPS-IH Caused by Nonsense Mutations
awarded by
University of Pennsylvania
2016 - 2019
New Nonsense Suppression Drugs to Treat MPS I
awarded by
National Institute of Neurological Disorders and Stroke/NIH/DHHS
2014 - 2017
Evaluation of Approved Drug Libraries for Translational Readthrough Activity
awarded by
Cystic Fibrosis Foundation
2013 - 2017
Suppression of the Idua-W402X Mutation in an MPS I-H Mouse
awarded by
National Institute of Neurological Disorders and Stroke/NIH/DHHS
2007 - 2013
Mechanism of Eukaryotic Translation Termination
awarded by
National Institute of General Medical Sciences/NIH/DHHS
2007 - 2012
Background
Education And Training
Bachelor of Science or Mathematics in Chemistry,
University of Alabama at Birmingham
1990
Contact
Full Name
Kim
Keeling