UNASSIGNED: A large number of @entity1 diseases are caused by nonsense mutations. These mutations result in premature protein termination and the expression of truncated, usually nonfunctional products. A promising therapeutic strategy for @entity1 suffering from premature termination codon (PTC)-mediated disorders is to suppress the nonsense mutation and restore the expression of the affected protein. Such a suppression approach using specific antibiotics and other read-through promoting agents has been shown to suppress PTCs and restore the production of several important proteins. Here, we report the establishment of a novel, rapid, and very efficient method for screening stop-codon read-through agents. We also show that, in both @entity1 cells and in a transgenic @entity19 model, distinct members of the @entity7646 antibiotic family can induce read-through of disease-causing stop codons leading to re-expression of several key proteins and to @entity146 phenotypes. Taken together, our results may help in the identification and characterization of well-needed customized pharmaceutical PTC suppression agents. KEY MESSAGES: Establishment of a flow cytometry-based reporter assay to identify nonsense mutation read-through agents. @entity7646 antibiotics can induce read-through of disease-causing stop codons. @entity7646 -induced protein restoration can alleviate disease-like phenotypes.

A flow cytometry-based reporter assay identifies XXXX antibiotics as nonsense mutation read-through agents.

@entity1 :: ('9606', 'Species') :: ['mammalian', 'human', 'patients'] @entity19 :: ('10090', 'Species') :: ['mouse'] @entity146 :: ('MESH:D001523', 'Disease') :: ['reduced disease'] @entity7646 :: ('CHEBI:25106', 'Chemical') :: ['macrolide', 'Macrolide']
@entity7646 :: (CHEBI:25106,Chemical) :: ['macrolide']
