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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)
,
Comprehensive Arthritis, Musculoskeletal, Bone and Autoimmunity Center (CAMBAC)
,
School of Medicine
Associate Scientist (C)
,
Cystic Fibrosis Research Center
,
School of Medicine
Assistant Professor (P)
,
Biochemistry & Molecular Genetics
,
Academic Joint Departments
Publications
Research
Teaching
Background
Contact
Publications
Selected Publications
Academic Article
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Year
Title
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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 mouse
.
Journal 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 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
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
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
.
Clinical 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 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
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.
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
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
Examining How CFTR Transcripts Are Targeted to NMD
awarded by
Cystic Fibrosis Foundation ^
Increasing CFTR Expression by NMD Attenuation
awarded by
Cystic Fibrosis Foundation ^
Investigating Nonsense-Mediated mRNA Decay as a Therapeutic Target
awarded by
Cystic Fibrosis Foundation ^
Investigating the Effects of Reducing Nonsense-Mediated mRNA Decay Efficiency
awarded by
NIH - OFFICE OF THE DIRECTOR
Private Grant
awarded by
NOVARTIS PHARMACEUTICALS CORPORATION
Private Grant
awarded by
Novartis Pharma AG
Private Grant
awarded by
ELOXX PHARMACEUTICALS
Private Grant
awarded by
ELOXX PHARMACEUTICALS
Private Grant
awarded by
ELOXX PHARMACEUTICALS
Investigator On
Amino Acids Incorporated at CFTR Nonsense Mutations During Readthrough
awarded by
Cystic Fibrosis Foundation ^
Evaluation of Approved Drug Libraries for Translational Readthrough Activity
awarded by
Cystic Fibrosis Foundation ^
Examining the Mechanism of a Readthrough Compound that Degrades eRF1
awarded by
Cystic Fibrosis Foundation ^
Exploring Nonsense Suppression as a Treatment for NF1
awarded by
GILBERT FAMILY FOUNDATION'S GENE THERAPY INITIATIVE, LLC (GTI)
Host Factors in Response to Therapeutic Monoclonal Antibodies and Vaccination
awarded by
National Institute of Allergy and Infectious Diseases/NIH/DHHS
Identify Drugs to Treat MPS-IH Caused by Nonsense Mutations
awarded by
University of Pennsylvania
Mechanism of Eukaryotic Translation Termination
awarded by
National Institute of General Medical Sciences/NIH/DHHS
New Nonsense Suppression Drugs to Treat MPS I
awarded by
National Institute of Neurological Disorders and Stroke/NIH/DHHS
Private Grant
awarded by
ZIKANI THERAPEUTICS, INC
Private Grant
awarded by
MONTE ROSA THERAPEUTICS AG
Suppression of the Idua-W402X Mutation in an MPS I-H Mouse
awarded by
National Institute of Neurological Disorders and Stroke/NIH/DHHS
UAB CF Research and Translation Core Center
awarded by
National Institute of Diabetes and Digestive and Kidney Diseases/NIH/DHHS
Teaching
Teaching Activities
GBSC720 - Journal Club 2 (Fall Term 2021)
GBSC720 - Journal Club 2 (Fall Term 2022)
GBSC720 - Journal Club 2 (Spring Term 2021)
GBSC720 - Journal Club 2 (Spring Term 2022)
GBSC720 - Journal Club 2 (Spring Term 2023)
Background
Education And Training
UAB Microbiology, Postdoctoral Fellowship
Bachelor of Science or Mathematics in Chemistry,
University of Alabama at Birmingham
1990
Contact
Full Name
Kim
Keeling