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标题:Enhanced thrombospondin-1 causes dysfunction of vascular endothelial cells derived from Fabry disease-induced pluripotent stem cells
时间:2020-07-26 19:53:21
DOI:10.1016/j.ebiom.2020.102633
PMID:31981984
大小:7018 kb
页数:16 PAGES
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目录:
  • Enhanced thrombospondin-1 causes dysfunction of vascular endothelial cells derived from Fabry disease-induced pluripotent stem cells
    • 1. Introduction
    • 2. Materials and Methods
      • 2.1. Generation of iPSCs from Fabry disease patients
      • 2.2. Bisulfite sequencing
      • 2.3. Differentiation of FD-iPSCs into vascular endothelial cells
      • 2.4. Tube-like structure assay in Matrigel
      • 2.5. Measurement of α-galactosidase activity
      • 2.6. Western blotting
      • 2.7. Immunostaining
      • 2.8. Quantitative RT-PCR
      • 2.9. FACS analysis
      • 2.10. Immunohistochemistry of renal tissues biopsied from FD patients
      • 2.11. RNA-Sequencing analysis
      • 2.12. Measurement of oxygen consumption rate
      • 2.13. Correction of GLA mutation in FD-iPSCs by CRISPR/Cas9
      • 2.14. Genetic ablation of TSP1 in FD-iPSCs by CRISPR/Cas9
      • 2.15. Targeted deep sequencing for analyzing sgRNA off-target effects in FD-iPSCs
      • 2.16. Statistical analysis
    • 3. Results
      • 3.1. Genetic mutations and clinical records for four FD patients
      • 3.2. Generation of FD-iPSCs
      • 3.3. Differentiation of FD-iPSCs into vascular endothelial cells
      • 3.4. Defective angiogenesis of FD-iPSC-derived vascular endothelial cells (FD-VECs)
      • 3.5. Aberrant expression of angiogenesis-associated factors in renal biopsies of FD patients
      • 3.6. Roles of SMAD2 signaling in the angiogenesis of FD-VECs
      • 3.7. Knockout of TSP-1 in FD-1-iPSCs using CRISPR/Cas9
      • 3.8. Correction of the mutated GLA gene using the CRISPR/Cas9 system in FD 1-iPSCs
      • 3.9. FD-VECs show altered transcriptional profiles in oxidative stress-related genes
    • 4. Discussion
    • Acknowledgments
    • Supplementary materials
      • References

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