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Figure 1

From: The pathogenesis linked to coenzyme Q10 insufficiency in iPSC-derived neurons from patients with multiple-system atrophy

Figure 1

Establishment and characterization of iPSCs from MSA patients and a healthy subject. (A) Coenzyme Q10 biosynthesis pathway. Coenzyme Q10 is composed of a benzoquinone and a decaprenyl side chain. COQ2, para-hydroxybenzoate (PHB)-polyprenyl transferase, catalyzes the condensation reaction of PHB and decaprenyl diphosphate. (B) Sequencing analysis of genomic DNA from iPSCs of each clone. MSA_A iPSCs harbored heterozygous mutations, c.1159C > T (p.R387*) and c.1178T > C (p.V393A), in exon 7 of the COQ2 gene (arrows). Three control lines and MSA_B lines did not have mutation in the COQ2 gene. (C) Representative morphology of iPSC colonies and immunochemical analysis of pluripotent stem cell markers, NANOG and OCT4. The scale bars represent 100 µm. Ho, Hoechst33258. (D) Karyotypes by the G-band staining method. Each clone shows a normal karyotype. (E) The lines showed tridermic markers (endoderm: AFP-positive cells, mesoderm: αSMA-positive cells, ectoderm: βIII-tubulin-positive cells). The scale bars represent 100 µm (endoderm and mesoderm) and 50 µm (ectoderm). β3tub, βIII-tubulin.

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