Journal Articles

Triamterene functions as an effective nonsense suppression agent for MPS I-H (Hurler Syndrome)

Siddiqui, A., Dundar, H., Sharma, J., Kaczmarczyk, A., Echols, J., Dai, Y., Sun, R., Du. M., Liu, Z., Zhao, R., Wood, T., Sanders, S., Rasmussen, L., Bostwick, R., Augelli-Szafran, C., Suto, M., Rowe, S., Bedwell, D., Keeling K.

International Journal of Molecular Sciences 24:4521 (2023).

Ataluren suppresses a premature termination codon in an MPS I-H mouse

Wang, D., Xue, X., Gunn, G., Du, M., Siddiqui, A., Weetall, M., Keeling, K.

Journal of Molecular Medicine (Berlin) 100:1223-1235 (2022).

A regulated NMD mouse model supports NMD inhibition as a viable therapeutic option to treat genetic diseases

Echols, J., Siddiqui, A., Dai, Y., Havasi, V., Sun, R., Kaczmarczyk, A., Keeling, K.

Disease Models & Mechanisms 13:dmm044891 (2020).

Identification of the Amino Acids Inserted During Suppression of CFTR Nonsense Mutations and Determination of Their Functional Consequences.

X. Xue, M. Mutyam, A. Thakerar, J. Mobley, R. Bridges, S. Rowe, K. Keeling, D. Bedwell.

Human Molecular Genetics, 26(16):3116-3129 (2017).

Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression.

B. Roy, W. Friesen, Y. Tomizawa, J. Leszyk, J. Zhuo, B. Johnson, J. Dakka, C. Trotta, X. Xue, V. Mutyam, K. Keeling, J. Mobley, S. Rowe, D. Bedwell, E. Welch, and A. Jacobson

PNAS, 113(44):12508-12513 (2016).

Discovery of clinically approved agents that promote suppression of CFTR nonsense mutations.

V. Muyam, M. Du, X. Xue, K. Keeling, L. White, R. Bostwick, L. Rasmussen, B. Liu, M.Mazur, J. Hong, E. Libby, F. Liang, H. Shang, M. Mense, M. Suto, D. Bedwell, S.

RoweAm J Respir Crit Care Med, 194(9): 1092-1103 (2016).

Long-term nonsense suppression therapy moderates MPS I-H disease progression.

G. Gunn, Y. Dai, M. Du, V. Belakhov, J. Kandasamy, T. Schoeb, T. Baasov, D. Bedwell, K. Keeling

Molecular Genetics and Metabolism 111:374-81 (2014).

Attenuation of nonsense-mediated mRNA decay enhances in vivo nonsense suppression.

K. Keeling, D. Wang, Y. Dai, S. Murugesan, B. Chenna, J. Clark, V. Belakhov, J. Kandasamy, S. Velu, T. Baasov, D. Bedwell.

PLoS One 8:e60478 (2013).

*This article is among the top 10% most cited PLoS One articles as of June 2017.

The novel synthetic aminoglycoside NB84 significantly attenuates biochemical defects associated with MPS I-H in the Idua-W392X mouse.

D. Wang, V. Belakhov, J. Kandasamy, T. Baasov, S. Li, Y. Li, D. Bedwell, and K. Keeling.

Molecular Genetics and Metabolism 105:116-125 (2012).

Enhancement of alveolar epithelial sodium channel activity with decreased cystic fibrosis transmembrane regulator expression in mouse lung

A. Lazrak, A. Jurkuvenaite, L. Chen, K. Keeling, J. Collawn, D. Bedwell, and S. Matalon

American Journal of Physiology-Lung Cellular and Molecular Physiology 301:L557-67 (2011).

Characterization of an MPS I-H Knock-In Mouse that Carries a Nonsense Mutation Analogous to the Human IDUA-W402X Mutation

D. Wang, C. Skukla, X. Liu, T. Schoeb, D. Bedwell, K. Keeling

Molecular Genetics and Metabolism 99:62-71 (2010).

Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a CF mouse model

M. Du, K. Keeling, L. Fan, X. Liu, D. Bedwell

Journal of Biological Chemistry 284:6885-92 (2009).

Distinct eRF3 Requirements Suggest Alternate eRF1 Conformations Mediate Peptide Release During Eukaryotic Translation Termination

H. Fan-Minogue, M. Du, A. Pisarev, A. Kallmeyer, J. Salas-Marco, K. Keeling, S. Thompson, T. Pestova, and D. Bedwell

Molecular Cell 30: 599-609 (2008).

eRF1 phosphorylaton by CK2 protein kinase is dynamic but has little effect on the efficiency of translation termination in Saccharomyces cerevisiae

A. Kallmeyer, K. Keeling, D. Bedwell

Eukaryotic Cell 5: 1378-1387 (2006).

Tpa1p is part of an mRNP complex that influences translation termination, mRNA deadenylation, and mRNA turnover in Saccharomyces cerevisiae

K. Keeling, J. Salas-Marco, L. Osherovich, D. Bedwell

Molecular and Cellular Biology 26: 5237- 5248 (2006).

Clinical doses of amikacin correct the CFTR-G542X stop mutation more efficiently than gentamicin in a transgenic CF mouse model

M. Du, K. Keeling, L. Fan, X. Liu, T. Kovacs, E. Sorscher, D. Bedwell

Journal of Molecular Medicine 84: 573-583 (2006).

Aminoglycosides as potential pharmacogenetic agents in the treatment of Hailey-Hailey disease

R. Kellermayer, R. Szigeti, K. Keeling, T. Bedekovics, D. Bedwell

Journal of Investigative Dermatology 126: 229-231 (2006).

Leaky termination at a premature stop codon antagonizes nonsense-mediated mRNA decay in S. cerevisiae 

K. Keeling, J. Lanier, A. Kaenjak-Angeletti, D. Bedwell 

RNA 10: 691-703 (2004).

Aminoglycoside suppression of a premature stop mutation in a Cftr-/- mouse carrying a human CFTR-G542X transgene

M. Du, J. Jones, J. Lanier, K. Keeling, J. Lindsey, A. Tousson, Z. Bebök, J. Whitsett, C. Rey, W. Colledge, M. Evans, E. Sorscher, D. Bedwell 

Journal of Molecular Medicine80: 595-604 (2002).

Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAs in a mammalian translation system              

K. Keeling, D. Bedwell  

Journal of Molecular Medicine 80: 367-376 (2002).

Gentamicin-mediated suppression of Hurler Syndrome stop mutations leads to partial restoration of a-L-iduronidase activity and a reduction of lysosomal glycosaminoglycan accumulation

K. Keeling, D. Brooks, J. Hopwood, P. Li, J. Thompson, D. Bedwell

Human Molecular Genetics10: 291-299 (2001).

Aminoglycoside antibiotics mediate context-dependent suppression of termination codons in a mammalian translation system

M. Manuvakhova, K. Keeling, D. Bedwell

RNA6: 1044-1055 (2000).

Lower dimer impurity incorporation may result in higher perfection of HEWL crystals grown in microgravity, a case study

D. Carter, K. Lim, J.  Ho, B. Wright, P. Twigg, T. Miller, J. Chapman, K. Keeling, J. Ruble, P. Vekilov, B. Thomas, F. Rosenberger, A. Chernov 

Journal of Protein Crystal Growth 196: 623-637 (1999).

PCAM: a multi-user facility-based protein crystallization apparatus for microgravity

D. Carter, B. Wright, T. Miller, J. Chapman, P. Twigg, K. Keeling, K. Moody, M. White, J. Click, J. Ruble, J. Ho, L. Adcock-Downey, T. Dowling, C. Chang, P. Ala, J. Rose, B.C. Wang, J. Declercq, C. Evrard, J. Rosenberg, J. Wery, D. Clawson, M. Wardell, W. Stallings, A. Stevens

Journal of Protein Crystal Growth 196: 610-622 (1999).

Diffusion-controlled crystallization apparatus for microgravity (DCAM):  flight and ground-based applications

D. Carter, B. Wright, T. Miller, J. Chapman, P. Twigg, K. Keeling, M. Whilte, J. Click, J.R. Ruble, J. Ho, L. Adcock-Downey, G. Bunick, J. Harp  

Journal of Crystal Growth 196: 602-609 (1999).

Interactions between an Fab fragment against gp41 of HIV-1 and its peptide epitope:  characterization using a peptide epitope library and molecular modeling

R. Singler, F. Ruker, D. Kattinger, G. Elliot, W. Hohne, P. Henklein, J. Ho, K. Keeling, D. Carter, E. Negel 

Protein Engineering 8: 471-479 (1995).

Fusion proteins as alternate crystallization paths to difficult structure problems   

D. Carter, F. Ruker, J. Ho, K. Keeling, G. Gilliland, X. Ji 

Protein and Peptide Letters 1: 175-178 (1994).

Three-dimensional structure of Schistosoma japonicum S-transferase fused wth a six-amino acid conserved neutralizing epitope of gp41 from HIV

K. Lim, J. Ho, K. Keeling, G. Gilliland, X. Ji, F. Ruker, D. Carter 

Protein Science 3: 2233-2244 (1994).

Preliminary crystallization studies of four crystal forms of serum albumin.

D. Carter, B. Chang, J. Ho, K. Keeling, Z. Krishnasami

European Journal of Biochemistry260: 1049-1052 (1994).

Reviews and Book Chapters

Nonsense Suppression as an Approach to Treat Lysosomal Storage Diseases

K. Keeling

Diseases 2016, 4(4), 32; doi:10.3390/diseases4040032.

Therapeutics Based on Stop Codon Readthrough

K. Keeling, X. Xue, G. Gunn, D. Bedwell

Annual Review of Genomics and Human Genetics 15: 371-94 (2014).

Suppression of Premature Termination Codons as a Therapeutic Approach

K. Keeling, D. Wang, S. Conard, D. Bedwell

Critical Reviews in Biochemistry and Molecular Biology 47:444-463 (2012)

Suppression of Nonsense Mutations as a Therapeutic Approach to Treat Genetic Diseases

K. Keeling and D. Bedwell

Wiley Interdisciplinary Reviews: RNA 2:837-52 (2011).

Recoding Therapies for Genetic Diseases

K. Keeling and D. Bedwell

Translational Recoding, pages 123-148.

R. Gesteland & J. Atkins, Eds., Springer Publications, New York, NY (2010).

Therapies of nonsense-associated diseases

K. Keeling, M. Du, D. Bedwell

Nonsense-Mediated mRNA Decay, pages 121-136.

L.E. Maquat, Ed., Landes Bioscience, Georgetown, TX (2006).

Pharmacological suppression of premature stop mutations that cause genetic diseases

K. Keeling and D. Bedwell

Current Pharmacogenomics 3: 259-269 (2005).