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- W2100254450 abstract "Diamond Blackfan anemia (DBA) is a genetic syndrome characterized by red blood cell aplasia in association with developmental abnormalities such as growth retardation, orofacial, hand or limb malformations, urogenital anomalies, and heart defects. The only known cause is heterozygosity for mutations in genes encoding ribosomal proteins. Understanding how defective ribosome biogenesis and function, important for all cells, causes defects in erythropoiesis and tissue-specific phenotypes during development is paramount to the evolution of effective treatment protocols. Here, we discuss how animal models based on mammals, insects, and fish replicate genetic or developmental aspects of DBA and have led to the identification of pathways and candidate molecules that are important in the pathogenesis of the disease. A recurring theme in many of these models suggests that defective ribosome biogenesis induces a p53-dependent cell cycle checkpoint in cells that require high levels of ribosome production and leads to cell type-specific, whole animal phenotypes." @default.
- W2100254450 created "2016-06-24" @default.
- W2100254450 creator A5050260893 @default.
- W2100254450 creator A5081106729 @default.
- W2100254450 date "2011-04-01" @default.
- W2100254450 modified "2023-09-25" @default.
- W2100254450 title "Animal Models of Diamond Blackfan Anemia" @default.
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- W2100254450 doi "https://doi.org/10.1053/j.seminhematol.2011.02.001" @default.
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