Journal
EXPERIMENTAL HEMATOLOGY
Volume 40, Issue 4, Pages 290-294Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.exphem.2011.12.003
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Funding
- National Institutes of Health [K08 DK075422, P30 CA147883, R01 HL31823, R01 AG033373, P20 RR016458]
- Ellison Medical Foundation [AG-IA-0052]
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Diamond-Blackfan anemia is a congenital hypoproliferative macrocytic anemia and 5q-syndrome myelodysplastic syndrome is an acquired hypoproliferative macrocytic anemia. Their common erythroid phenotype reflects a shared pathophysiology-haploinsufficiency of one of many ribosomal proteins and somatic deletion of one allele of the ribosomal protein S14 gene, respectively. Although these abnormalities lead to defective ribosome biogenesis, why ribosomal protein hemizygosity results in anemia is not certain. Here, we characterize the hematopoietic phenotype of mice lacking one allele of the ribosomal protein S6 gene. The mice have an erythroid phenotype similar to both Diamond-Blackfan anemia and the 5q- syndrome and lenalidomide therapy improves their anemia. (C) 2012 ISEH - Society for Hematology and Stem Cells. Published by Elsevier Inc.
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