Glucocorticoids improve erythroid progenitor maintenance and dampen Trp53 response in a mouse model of Diamond-Blackfan anaemia

Glucocorticoids improve erythroid progenitor maintenance and dampen Trp53 response in a mouse model of Diamond-Blackfan anaemia, Sjögren SE, Siva K, Soneji S, et al.,

Br J Haematol. 2015 Aug 25.

Majority of the patients with Diamond-Blackfan anaemia (DBA) are responsive to glucocorticoid treatment. However, the therapeutic mechanisms of glucocorticoids in DBA remain largely unknown. In a ribosomal protein s19 (Rps19) deficient mouse model of DBA, glucocorticoid treatment was demonstrated to reduce apoptosis, rescue erythroid progenitor depletion and premature differentiation of erythroid cells. Additionally, glucocorticoids were found to prevent Trp53 activation in Rps19-deficient cells- in a disease-specific manner. The study is important for enlightening the mechanism of action of glucocorticoids in DBA for the first time.

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