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Pluripotent stem cell model of Shwachman–Diamond syndrome reveals apoptotic predisposition of hemoangiogenic progenitors

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...

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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7481313/
https://www.ncbi.nlm.nih.gov/pubmed/32908229
http://dx.doi.org/10.1038/s41598-020-71844-8