Downregulation of Securin by the variant RNF213 R4810K (rs112735431, G>A) reduces angiogenic activity of induced pluripotent stem cell-derived vascular endothelial cells from moyamoya patients

• Published in: Biochemical and biophysical research communications Vol. 438; no. 1; pp. 13 - 19
• Main Authors: Hitomi, Toshiaki, Habu, Toshiyuki, Kobayashi, Hatasu, Okuda, Hiroko, Harada, Kouji H., Osafune, Kenji, Taura, Daisuke, Sone, Masakatsu, Asaka, Isao, Ameku, Tomonaga, Watanabe, Akira, Kasahara, Tomoko, Sudo, Tomomi, Shiota, Fumihiko, Hashikata, Hirokuni, Takagi, Yasushi, Morito, Daisuke, Miyamoto, Susumu, Nakao, Kazuwa, Koizumi, Akio
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 16.08.2013
• Subjects: 60 APPLIED LIFE SCIENCES; Adenosine Triphosphatases; alleles; ANGIOGENESIS; CARRIERS; Cells, Cultured; Child; DISEASES; Down-Regulation; endothelial cells; Endothelial Cells - metabolism; Endothelial Cells - pathology; ENDOTHELIUM; Female; FIBROBLASTS; gene expression; GENES; Humans; IN VITRO; induced pluripotent stem cells; INHIBITION; iPS cells; Male; Middle Aged; MITOSIS; Moyamoya disease; Moyamoya Disease - metabolism; Moyamoya Disease - pathology; Mutation - genetics; Neoplasm Proteins - metabolism; Neovascularization, Pathologic - pathology; Neovascularization, Pathologic - physiopathology; PATIENTS; Pluripotent Stem Cells - metabolism; Pluripotent Stem Cells - pathology; RNA; RNA interference; RNF213; Securin; STEM CELLS; Ubiquitin-Protein Ligases - genetics; Ubiquitin-Protein Ligases - metabolism; VEINS; Angiogenesis; Moyamoya disease; RNF213; Securin; iPS cells; Endothelium

•Angiogenic activities were reduced in iPSECs from MMD patients.•Many mitosis-regulated genes were downregulated in iPSECs from MMD patients.•RNF213 R4810K downregulated Securin and inhibited angiogenic activity.•Securin suppression by siRNA reduced angiogenic activities of iPSECs and HUVECs. Moyamo...