In order to better understand the effects of AC1903 on G418 uptake and PTC readthrough, we exposed DMS-114 cells and JEB01 keratinocytes, derived from a junctional epidermolysis bullosa (JEB) patient with a homozygous nonsense mutation (p.R688X) in the COL17A1 gene, to increasing concentrations of G418 without or with pretreatment with 150 μM AC1903. This evidence concerns the gene COL17A1 and Junctional epidermolysis bullosa, Herlitz type.