Fig. 1

Generation of hiPSCs using peripheral blood mononuclear cells derived from patients with Fabry disease nephropathy. A Light and electron microscopic findings of renal tissue biopsies from FD patients. On microscopic examination, there are some vague vacuolizations (black arrow) of podocytes (upper figure, H&E stain, ×400). Multi-lamellated myelin figures (red arrow), so-called zebra bodies as typical findings of FDN, are found in electron microscopy (lower figure). B Sequencing results of mutation in GLA gene from FD patients. C Schematic of iPSC reprogramming from patient by Sendai virus. D Immunofluorescence staining of three stem cell proteins (NANOG, SSEA-4, TRA-1-81). Scale bar = 50 μm, E Flow cytometry analysis of pluripotency markers (NANOG, SSEA-4 TRA-1-81) in cells. F Immunofluorescence staining of three germ layer markers: PAX4 for detecting ectoderm differentiation, SM22A for detecting mesoderm differentiation, and FOX2A for detecting endoderm differentiation. Scale bar = 50 μm. hiPSC, human pluripotent stem cell; FDN, Fabry disease nephropathy