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Fig. 1 | Molecular Neurodegeneration

Fig. 1

From: Premature termination codon readthrough upregulates progranulin expression and improves lysosomal function in preclinical models of GRN deficiency

Fig. 1

Induction of PTC readthrough by G418 and CDX5 enhancers in cells expressing GRN-V5. a Schematic of full-length PGRN highlighting the position of the S116X (UAA), R418X (UGA), and R493X (UGA) nonsense mutations in relation to the position of individual granulin peptides and the C-terminal V5 tag. b HEK293 cell lines stably expressing GRN-V5 with the indicated nonsense mutations were treated with G418 and the indicated concentrations of CDX5–1, CDX5–196, and CDX5–288 for 72 h. Cell culture supernatants (extracellular) and cell lysates (intracellular) were subjected to automated capillary electrophoresis western analysis. Full-length PGRN was detected with a V5 antibody. Actin was measured in cell lysates as a loading control. The readthrough enhancement ratios are indicated under the lanes. The proportion loaded was 15–20 fold lower for the extracellular samples than for the intracellular samples

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Molecular Neurodegeneration

ISSN: 1750-1326