Attenuation of Nonsense-Mediated mRNA Decay Enhances In Vivo Nonsense Suppression

Suppression of premature termination codons as a therapeutic approach

(2012) Kim M. Keeling et al.   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

Nonsense-mediated mRNA decay (NMD) in animal embryogenesis: to die or not to die, that is the question

(2011) Jungwook Hwang et al.   CURRENT OPINION IN GENETICS & DEVELOPMENT

Chronic ataluren (PTC124) treatment of nonsense mutation cystic fibrosis

(2011) M. Wilschanski et al.   EUROPEAN RESPIRATORY JOURNAL

RNA Homeostasis Governed by Cell Type-Specific and Branched Feedback Loops Acting on NMD

(2011) Lulu Huang et al.   MOLECULAR CELL

The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse

(2011) Dan Wang et al.   MOLECULAR GENETICS AND METABOLISM

N- and C-terminal Upf1 phosphorylations create binding platforms for SMG-6 and SMG-5:SMG-7 during NMD

(2011) Yukiko Okada-Katsuhata et al.   NUCLEIC ACIDS RESEARCH

Perturbation of thymocyte development in nonsense-mediated decay (NMD)-deficient mice

(2011) P. A. Frischmeyer-Guerrerio et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Autoregulation of the nonsense-mediated mRNA decay pathway in human cells

(2011) H. Yepiskoposyan et al.   RNA

Suppression of nonsense mutations as a therapeutic approach to treat genetic diseases

(2011) Kim M. Keeling et al.   Wiley Interdisciplinary Reviews-RNA

Ataluren (PTC124) Induces Cystic Fibrosis Transmembrane Conductance Regulator Protein Expression and Activity in Children with Nonsense Mutation Cystic Fibrosis

(2010) Isabelle Sermet-Gaudelus et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Repairing faulty genes by aminoglycosides: Development of new derivatives of geneticin (G418) with enhanced suppression of diseases-causing nonsense mutations

(2010) Igor Nudelman et al.   BIOORGANIC & MEDICINAL CHEMISTRY

Heritability in the Efficiency of Nonsense-Mediated mRNA Decay in Humans

(2010) Cathal Seoighe et al.   PLoS One

Smg1 is required for embryogenesis and regulates diverse genes via alternative splicing coupled to nonsense-mediated mRNA decay

(2010) D. R. McIlwain et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation

(2009) Dan Wang et al.   MOLECULAR GENETICS AND METABOLISM

NMD is essential for hematopoietic stem and progenitor cells and for eliminating by-products of programmed DNA rearrangements

(2008) J. Weischenfeldt et al.   GENES & DEVELOPMENT

A meta-analysis of nonsense mutations causing human genetic disease

(2008) Matthew Mort et al.   HUMAN MUTATION

PTC124 induces CFTR full-length production and activity in children with nonsense-mutation-mediated CF

(2008) I. Sermet-Gaudelus et al.   Journal of Cystic Fibrosis

PTC124 treatment over 3 months improves pharmacodynamic and clinical parameters in patients with nonsense-mutation-mediated CF

(2008) M. Wilschanski et al.   Journal of Cystic Fibrosis

Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial

(2008) Eitan Kerem et al.   LANCET

The multiple lives of NMD factors: balancing roles in gene and genome regulation

(2008) Olaf Isken et al.   NATURE REVIEWS GENETICS

Introducing sense into nonsense in treatments of human genetic diseases

(2008) Liat Linde et al.   TRENDS IN GENETICS