Pluripotent stem cell model of Shwachman-Diamond syndrome reveals apoptotic predisposition of hemoangiogenic progenitors was written by Hamabata, Takayuki;Umeda, Katsutsugu;Kouzuki, Kagehiro;Tanaka, Takayuki;Daifu, Tomoo;Nodomi, Seishiro;Saida, Satoshi;Kato, Itaru;Baba, Shiro;Hiramatsu, Hidefumi;Osawa, Mitsujiro;Niwa, Akira;Saito, Megumu K.;Kamikubo, Yasuhiko;Adachi, Souichi;Hashii, Yoshiko;Shimada, Akira;Watanabe, Hiroyoshi;Osafune, Kenji;Okita, Keisuke;Nakahata, Tatsutoshi;Watanabe, Kenichiro;Takita, Junko;Heike, Toshio. And the article was included in Scientific Reports in 2020.SDS of cas: 63208-82-2 The following contents are mentioned in the article:
Shwachman-Diamond syndrome (SDS), an autosomal recessive disorder characterized by bone marrow failure, exocrine pancreatic insufficiency, and skeletal abnormalities, is caused by mutations in the Shwachman-Bodian-Diamond syndrome (SBDS) gene, which plays a role in ribosome biogenesis. Although the causative genes of congenital disorders frequently involve regulation of embryogenesis, the role of the SBDS gene in early hematopoiesis remains unclear, primarily due to the lack of a suitable exptl. model for this syndrome. In this study, we established induced pluripotent stem cells (iPSCs) from patients with SDS (SDS-iPSCs) and analyzed their in vitro hematopoietic and endothelial differentiation potentials. SDS-iPSCs generated hematopoietic and endothelial cells less efficiently than iPSCs derived from healthy donors, principally due to the apoptotic predisposition of KDR+CD34+ common hemoangiogenic progenitors. By contrast, forced expression of SBDS gene in SDS-iPSCs or treatment with a caspase inhibitor reversed the deficiency in hematopoietic and endothelial development, and decreased apoptosis of their progenitors, mainly via p53-independent mechanisms. Patient-derived iPSCs exhibited the hematol. abnormalities associated with SDS even at the earliest hematopoietic stages. These findings will enable us to dissect the pathogenesis of multiple disorders associated with ribosomal dysfunction. This study involved multiple reactions and reactants, such as 2-(2-Imino-4,5,6,7-tetrahydrobenzothiazol-3-yl)-1-p-tolylethanone Hydrobromide (cas: 63208-82-2SDS of cas: 63208-82-2).
2-(2-Imino-4,5,6,7-tetrahydrobenzothiazol-3-yl)-1-p-tolylethanone Hydrobromide (cas: 63208-82-2) belongs to thiazole derivatives. Thiazole rings are planar and aromatic. Thiazoles are characterized by larger pi-electron delocalization than the corresponding oxazoles and have therefore greater aromaticity. Various laboratory methods exist for the organic synthesis of thiazoles. Prominent is the Hantzsch thiazole synthesis is a reaction between haloketones and thioamides.SDS of cas: 63208-82-2
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Thiazole | C3H3NS – PubChem,
Thiazole | chemical compound | Britannica