Skip to main content
Fig. 6 | Molecular Neurodegeneration

Fig. 6

From: Trehalose upregulates progranulin expression in human and mouse models of GRN haploinsufficiency: a novel therapeutic lead to treat frontotemporal dementia

Fig. 6

Trehalose rescues PGRN deficiency in induced pluripotent stem cell (iPSC) embryoid bodies (EBs) and iPSC-derived neurons. a Immunofluorescence verifies that iPSC colonies generated from control and GRN-mutation patients express the following standard pluripotent stem-cell markers: pluripotent transcription factors Oct4, Nanog, and Sox2 (green) and surface markers SSEA4, TRA 1–81, and TRA 1–60 (red). b Representative phase images of embryoid body (EB) formation during differentiation and highly pure iPSC-derived neurons approximately 1-week post plating. c Immunoblot of embryoid body cell lysates generated from one CTL and one GRN iPSC lines after treatment with vehicle, trehalose (100 mM), or PP242 (1 μM) for 24 h. d Representative Immunoblot of cell lysates from GRN iPSC-derived neurons treated with trehalose (100 mM) for 24 h. Two independent replicates from the GRN iPSC-neurons are shown. An untreated control (CTL) patient iPSC-neuron lysate was included to show relative differences in PGRN expression levels. e Quantification of PGRN expression from vehicle and trehalose treated GRN iPSC-neurons (n = 5 independent experiments for each). In all graphs, the bars represent the mean ± SEM. ***Differs from control, P < 0.001 using unpaired two-tailed Student’s t-test

Back to article page
\