FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2618650759> ?p ?o ?g. }

• W2618650759 endingPage "3129" @default.
• W2618650759 startingPage "3116" @default.
• W2618650759 abstract "In-frame premature termination codons (PTCs) account for ∼11% of all disease-associated mutations. PTC suppression therapy utilizes small molecules that suppress translation termination at a PTC to restore synthesis of a full-length protein. PTC suppression is mediated by the base pairing of a near-cognate aminoacyl-tRNA with a PTC and subsequently, the amino acid becomes incorporated into the nascent polypeptide at the site of the PTC. However, little is known about the identity of the amino acid(s) inserted at a PTC during this process in mammalian cells, or how the surrounding sequence context influences amino acid incorporation. Here, we determined the amino acids inserted at the cystic fibrosis transmembrane conductance regulator (CFTR) W1282X PTC (a UGA codon) in the context of its three upstream and downstream CFTR codons during G418-mediated suppression. We found that leucine, cysteine and tryptophan are inserted during W1282X suppression. Interestingly, these amino acids (and their proportions) are significantly different from those recently identified following G418-mediated suppression of the CFTR G542X UGA mutation. These results demonstrate for the first time that local mRNA sequence context plays a key role in near-cognate aminoacyl-tRNA selection during PTC suppression. We also found that some variant CFTR proteins generated by PTC suppression exhibit reduced maturation and activity, indicating the complexity of nonsense suppression therapy. However, both a CFTR corrector and potentiator enhanced activity of protein variants generated by G418-mediated suppression. These results suggest that PTC suppression in combination with CFTR modulators may be beneficial for the treatment of CF patients with PTCs." @default.
• W2618650759 created "2017-06-05" @default.
• W2618650759 creator A5000385368 @default.
• W2618650759 creator A5004264403 @default.
• W2618650759 creator A5033315508 @default.
• W2618650759 creator A5033410717 @default.
• W2618650759 creator A5054277797 @default.
• W2618650759 creator A5060475091 @default.
• W2618650759 creator A5064807890 @default.
• W2618650759 creator A5081232198 @default.
• W2618650759 date "2017-05-31" @default.
• W2618650759 modified "2023-10-01" @default.
• W2618650759 title "Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences" @default.
• W2618650759 cites W1517922421 @default.
• W2618650759 cites W1589654967 @default.
• W2618650759 cites W1653043221 @default.
• W2618650759 cites W178332538 @default.
• W2618650759 cites W1924365648 @default.
• W2618650759 cites W1984653141 @default.
• W2618650759 cites W1988397255 @default.
• W2618650759 cites W1991977085 @default.
• W2618650759 cites W1994621182 @default.
• W2618650759 cites W1997504014 @default.
• W2618650759 cites W2003041746 @default.
• W2618650759 cites W2015556811 @default.
• W2618650759 cites W2018768650 @default.
• W2618650759 cites W2035131077 @default.
• W2618650759 cites W2045716945 @default.
• W2618650759 cites W2049734985 @default.
• W2618650759 cites W2062814567 @default.
• W2618650759 cites W2066841033 @default.
• W2618650759 cites W2067786219 @default.
• W2618650759 cites W2069289373 @default.
• W2618650759 cites W2071226544 @default.
• W2618650759 cites W2071407356 @default.
• W2618650759 cites W2078078890 @default.
• W2618650759 cites W2080281443 @default.
• W2618650759 cites W2085811948 @default.
• W2618650759 cites W2092985615 @default.
• W2618650759 cites W2097058336 @default.
• W2618650759 cites W2106654613 @default.
• W2618650759 cites W2112078820 @default.
• W2618650759 cites W2114594888 @default.
• W2618650759 cites W2121996960 @default.
• W2618650759 cites W2126330038 @default.
• W2618650759 cites W2128903674 @default.
• W2618650759 cites W2131056651 @default.
• W2618650759 cites W2138022079 @default.
• W2618650759 cites W2140868856 @default.
• W2618650759 cites W2141439233 @default.
• W2618650759 cites W2158288696 @default.
• W2618650759 cites W2158501031 @default.
• W2618650759 cites W2172058029 @default.
• W2618650759 cites W2269683575 @default.
• W2618650759 cites W2467866301 @default.
• W2618650759 cites W2528039416 @default.
• W2618650759 cites W2528751872 @default.
• W2618650759 cites W2549271860 @default.
• W2618650759 cites W2558608270 @default.
• W2618650759 cites W2562038667 @default.
• W2618650759 cites W4241884576 @default.
• W2618650759 doi "https://doi.org/10.1093/hmg/ddx196" @default.
• W2618650759 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5886253" @default.
• W2618650759 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/28575328" @default.
• W2618650759 hasPublicationYear "2017" @default.
• W2618650759 type Work @default.
• W2618650759 sameAs 2618650759 @default.
• W2618650759 citedByCount "65" @default.
• W2618650759 countsByYear W26186507592017 @default.
• W2618650759 countsByYear W26186507592018 @default.
• W2618650759 countsByYear W26186507592019 @default.
• W2618650759 countsByYear W26186507592020 @default.
• W2618650759 countsByYear W26186507592021 @default.
• W2618650759 countsByYear W26186507592022 @default.
• W2618650759 countsByYear W26186507592023 @default.
• W2618650759 crossrefType "journal-article" @default.
• W2618650759 hasAuthorship W2618650759A5000385368 @default.
• W2618650759 hasAuthorship W2618650759A5004264403 @default.
• W2618650759 hasAuthorship W2618650759A5033315508 @default.
• W2618650759 hasAuthorship W2618650759A5033410717 @default.
• W2618650759 hasAuthorship W2618650759A5054277797 @default.
• W2618650759 hasAuthorship W2618650759A5060475091 @default.
• W2618650759 hasAuthorship W2618650759A5064807890 @default.
• W2618650759 hasAuthorship W2618650759A5081232198 @default.
• W2618650759 hasBestOaLocation W26186507591 @default.
• W2618650759 hasConcept C104317684 @default.
• W2618650759 hasConcept C105580179 @default.
• W2618650759 hasConcept C149364088 @default.
• W2618650759 hasConcept C151730666 @default.
• W2618650759 hasConcept C153911025 @default.
• W2618650759 hasConcept C153957851 @default.
• W2618650759 hasConcept C164718446 @default.
• W2618650759 hasConcept C2777893369 @default.
• W2618650759 hasConcept C2778428886 @default.
• W2618650759 hasConcept C2779343474 @default.
• W2618650759 hasConcept C501734568 @default.
• W2618650759 hasConcept C515207424 @default.
• W2618650759 hasConcept C54355233 @default.

• next