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Article

Effects of gentamicin inducing readthrough premature stop Codons: A study of alpha-L-iduronidase nonsense variants in COS-7 Cells.

Biochemical and biophysical research communications - 25 Dec 2022

Ngiwsara Lukana, Sawangareetrakul Phannee, Wattanasirichaigoon Duangrurdee, Tim-Aroon Thipwimol, Dejkhamron Prapai, Champattanachai Voraratt, Ketudat-Cairns James R, Svasti Jisnuson

Abstract excerpt

Mucopolysaccharidosis type I Hurler syndrome (MPS IH) is a severe lysosomal storage disorder caused by alpha-l-iduronidase (IDUA) deficiency. Premature truncation mutations (PTC) are the most common (50%-70%) type of IDUA mutations and correlate with MPS IH. Nonsense suppression therapy is a therapeutic approach that aims to induce stop codon readthrough. The different ability of gentamicin to bind mutant mRNA in...

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PMID: 36332477
DOI: 10.1016/j.bbrc.2022.10.081

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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