DBA 缺陷：远不止表面所见。
Defects in DBA: more than meets the eye.
机构信息
Hôpital Universitaire Robert-Debré.
出版信息
Blood. 2013 Aug 8;122(6):856-7. doi: 10.1182/blood-2013-06-508465.
PMID:23929834
Abstract
In this issue of Blood, Garçon et al describe successful derivation of induced pluripotent stem cells (iPSs) from fibroblasts of Diamond Blackfan anemia (DBA) patients with 2 distinct ribosomal defects. Using these cells, the authors showed that they not only exhibit defective erythropoiesis but also globally impaired hematopoiesis affecting multipotent progenitors.
摘要
在本期《Blood》中，Garçon 等人描述了从具有 2 种不同核糖体缺陷的 Diamond Blackfan 贫血（DBA）患者的成纤维细胞中成功衍生诱导多能干细胞（iPS）。作者利用这些细胞表明，它们不仅表现出红细胞生成缺陷，而且还普遍影响多能祖细胞的造血功能受损。
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