David M Bedwell Selected Research

Nonsense Codon (Nonsense Mutation)

11/2016	Discovery of Clinically Approved Agents That Promote Suppression of Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations.
4/2014	Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor.
3/2014	Long-term nonsense suppression therapy moderates MPS I-H disease progression.
1/2014	Therapeutics based on stop codon readthrough.
1/2013	Attenuation of nonsense-mediated mRNA decay enhances in vivo nonsense suppression.
1/2012	The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse.
11/2011	Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases.
3/2009	Poly-L-aspartic acid enhances and prolongs gentamicin-mediated suppression of the CFTR-G542X mutation in a cystic fibrosis mouse model.
2/2008	PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model.
6/2002	Clinically relevant aminoglycosides can suppress disease-associated premature stop mutations in the IDUA and P53 cDNAs in a mammalian translation system.

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David M Bedwell Research Topics

Disease

9	Inborn Genetic Diseases (Disease, Hereditary) 06/2020 - 06/2002
8	Cystic Fibrosis (Mucoviscidosis) 01/2021 - 09/2002
3	Mucopolysaccharidosis I (Hurler Syndrome) 03/2014 - 06/2002
3	Lysosomal Storage Diseases (Lysosomal Storage Disease) 03/2014 - 06/2002
2	Neurofibromatosis 1 (Neurofibromatosis Type I) 01/2022 - 06/2020
2	Neoplasms (Cancer) 06/2020 - 06/2002
1	Starvation 05/2015
1	Ototoxicity 04/2014
1	Disease Progression 03/2014
1	Human Influenza (Influenza) 09/2013
1	Infections 09/2013
1	Benign Familial Pemphigus (Hailey Hailey Disease) 01/2006

Drug/Important Bio-Agent (IBA)

10	Nonsense Codon (Nonsense Mutation)IBA 11/2016 - 06/2002
5	Cystic Fibrosis Transmembrane Conductance Regulator (Protein, CFTR)IBA 11/2016 - 09/2002
3	AminoglycosidesIBA 11/2011 - 06/2002
2	Neurofibromin 1 (Neurofibromin)IBA 01/2022 - 06/2020
2	Proteins (Proteins, Gene)FDA Link 01/2022 - 05/2015
2	Terminator Codon (Termination Codon)IBA 01/2021 - 02/2008
2	Gentamicins (Gentamicin)FDA LinkGeneric 04/2014 - 03/2009
2	Iduronidase (alpha-L-Iduronidase)IBA 03/2014 - 01/2012
1	Deubiquitinating EnzymesIBA 05/2015
1	NitrogenIBA 05/2015
1	RNA (Ribonucleic Acid)IBA 05/2015
1	EndonucleasesIBA 01/2014
1	Interleukin-6 (Interleukin 6)IBA 09/2013
1	AnionsIBA 09/2013
1	Clodronic Acid (Clodronate)IBA 09/2013
1	Liposomes (Liposome)IBA 09/2013
1	GlycosaminoglycansIBA 01/2012
1	Aspartic Acid (Aspartate)FDA Link 03/2009
1	atalurenIBA 02/2008

Therapy/Procedure

7	Therapeutics 06/2020 - 06/2002