PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 23027640-2 2012 Here, we compare for the first time the relative efficacies of new-generation aminoglycosides NB30, NB54 and the chemical compound PTC124 on retinal toxicity and read-through efficacy of a nonsense mutation in the USH1C gene, which encodes the scaffold protein harmonin. Aminoglycosides 78-93 USH1 protein network component harmonin Homo sapiens 214-219 24664766-4 2014 Currently only the amelioration of the hearing deficiency is implemented, but no treatment of the senso-neuronal degeneration in the eye exists.In our studies we are focusing on the evaluation of gene-based therapies to cure the retinal degeneration of USH1C patients: (i) gene augmentation using recombinant adeno-associated virus, (ii) genome editing by homologous recombination mediated by zinc-finger nucleases and, (iii) read-through therapy using novel designer aminoglycosides and PTC124. Aminoglycosides 468-483 USH1 protein network component harmonin Homo sapiens 253-258 20671281-0 2010 Beneficial read-through of a USH1C nonsense mutation by designed aminoglycoside NB30 in the retina. Aminoglycosides 65-79 USH1 protein network component harmonin Homo sapiens 29-34 20671281-6 2010 Here, we investigated the capability of the novel synthetic aminoglycoside NB30 for the translational read-through of the USH1C-p.R31X nonsense mutation as a retinal therapy option. Aminoglycosides 60-74 USH1 protein network component harmonin Homo sapiens 122-127 20671281-14 2010 CONCLUSIONS: Commercial aminoglycosides and NB30 induced significant read-through of the USH1C-p.R31X nonsense mutation. Aminoglycosides 24-39 USH1 protein network component harmonin Homo sapiens 89-94