Results 21 to 30 of about 12,504 (200)

Variable readthrough responsiveness of nonsense mutations in hemophilia A

open access: yesHaematologica, 2020
Readthrough therapy relies on the use of small molecules that enable premature termination codons in mRNA open reading frames to be misinterpreted by the translation machinery, thus allowing the generation of full-length, potentially functional proteins ...
Lluis Martorell   +4 more
doaj   +4 more sources

Deciphering the molecular mechanism of stop codon readthrough [PDF]

open access: yesBiological Reviews, 2020
Recognition of the stop codon by the translation machinery is essential to terminating translation at the right position and to synthesizing a protein of the correct size.
Martine Palma, F. Lejeune
semanticscholar   +4 more sources

The unfolded protein response affects readthrough of premature termination codons

open access: yesEMBO Molecular Medicine, 2014
One‐third of monogenic inherited diseases result from premature termination codons (PTCs). Readthrough of in‐frame PTCs enables synthesis of full‐length functional proteins.
Steven M Rowe, Msph   +2 more
exaly   +2 more sources

Pre-mRNA processing factors differentially impact coordination between co-transcriptional cleavage and transcription termination [PDF]

open access: yesNature Communications
Co-transcriptional cleavage and transcription termination are closely related processes during mRNA maturation, yet their coordination remains poorly understood due to difficulties in detecting these transient events.
Xianhao Jin   +16 more
doaj   +2 more sources

Cellular senescence limits translational readthrough [PDF]

open access: yesBiology Open, 2021
ABSTRACT The origin and evolution of cancer cells is considered to be mainly fueled by DNA mutations. Although translation errors could also expand the cellular proteome, their role in cancer biology remains poorly understood. Tumor suppressors called caretakers block cancer initiation and progression by preventing DNA mutations and ...
Neylen del Toro   +10 more
openaire   +3 more sources

Drug-induced eRF1 degradation promotes readthrough and reveals a new branch of ribosome quality control.

open access: yesCell Reports, 2023
Suppression of premature termination codons (PTCs) by translational readthrough is a promising strategy to treat a wide variety of severe genetic diseases caused by nonsense mutations.
Lukas-Adrian Gurzeler   +18 more
semanticscholar   +2 more sources

Mechanisms of readthrough mitigation reveal principles of GCN1-mediated translational quality control

open access: yesCell, 2023
Summary Readthrough into the 3′ untranslated region (3′ UTR) of the mRNA results in the production of aberrant proteins. Metazoans efficiently clear readthrough proteins, but the underlying mechanisms remain unknown.
Martin B D Müller   +3 more
semanticscholar   +1 more source

Translation readthrough mitigation [PDF]

open access: yesNature, 2016
A fraction of ribosomes engaged in translation will fail to terminate when reaching a stop codon, yielding nascent proteins inappropriately extended on their C termini. Although such extended proteins can interfere with normal cellular processes, known mechanisms of translational surveillance are insufficient to protect cells from potential dominant ...
Arribere, Joshua A   +6 more
openaire   +4 more sources

Statistical analysis of readthrough levels for nonsense mutations in mammalian cells reveals a major determinant of response to gentamicin. [PDF]

open access: yesPLoS Genetics, 2012
The efficiency of translation termination depends on the nature of the stop codon and the surrounding nucleotides. Some molecules, such as aminoglycoside antibiotics (gentamicin), decrease termination efficiency and are currently being evaluated for ...
Célia Floquet   +3 more
doaj   +1 more source

Genome-scale quantification and prediction of pathogenic stop codon readthrough by small molecules

open access: yesbioRxiv, 2023
Premature termination codons (PTCs) cause ∼10-20% of Mendelian diseases and are the major mechanism of tumor suppressor gene inactivation in cancer.
Ignasi Toledano, F. Supek, Ben Lehner
semanticscholar   +1 more source

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