xanthine oxidase inhibitors br Resource table Resource detai

Resource table Resource details Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder commonly caused by heterozygous mutations in the transforming growth factor beta receptors (TGFBR)-1 or TGFBR2 (Loeys et al., 2005, 2006). In the current study, peripheral blood mononuclear xanthine oxidase inhibitors (PBMCs) were collected from a 38-year-old female LDS patient with a R193W mutation in the TGFBR2 gene. Patient PBMCs were reprogrammed into induced pluripotent stem cells (iPSCs) by electroporation with five episomal plasmids encoding human OCT4, SOX2, MYC2, KLF4, and BCL-X. The generated iPSCs displayed typical human embryonic stem cell-like colony morphology and growth characteristics (Fig. 1A). The iPSCs stained positive for alkaline phosphatase activity (Fig. 1B). Expression of the pluripotency markers NANOG, OCT4, SOX2, and TRA-1-60 were confirmed by immunofluorescence staining (Fig. 1C). Moreover, the presence of TGFBR2 mutation (R193W) in the iPSC line was confirmed by sequencing (Fig. 1D). Integration analysis shown that the episomal plasmids used for reprogramming did not integrate into the iPSC genome (Fig. 1F). In addition, the iPSCs presented a normal karyotype (46, XY) (Fig. 1E) after ten culture passages and free of mycoplasma (Supplementary Fig. 1). An in vivo teratoma formation assay further verified the pluripotent nature of the derived iPSC line (Fig. 1G).
Materials and methods
Acknowledgments We thank Dr. Tong Yang for skillful technical assistance. We also thank the following organizations for financial support: National Natural Science Foundation of China (Grant No. 81570422, 81500194), Youth Foundation of Guizhou Provincial People\'s Hospital (Grant No. GZSYQN [2016]03), Youth Foundation of Zhongshan Hospital (Grant No. 2015ZSQN48), Talent Training Program Foundation for the Excellent Youth Supported by Zhongshan Hospital (Grant No. 2015ZSYXQN12), Joint Project Funding for Major Diseases in Shanghai (Grant No. 2014ZYJB0402). The following are the supplementary data related to this article.