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 Blood, 5 March 2009, Vol. 113, No. 10, pp. 2136-2144.
Prepublished online as a Blood First Edition Paper on December 24, 2008; DOI 10.1182/blood-2008-09-115238.

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REVIEW ARTICLE

Chaperoning erythropoiesis

Mitchell J. Weiss1, and Camila O. dos Santos1

1 Division of Hematology, The Children's Hospital of Philadelphia, PA

Multisubunit complexes containing molecular chaperones regulate protein production, stability, and degradation in virtually every cell type. We are beginning to recognize how generalized and tissue-specific chaperones regulate specialized aspects of erythropoiesis. For example, chaperones intersect with erythropoietin signaling pathways to protect erythroid precursors against apoptosis. Molecular chaperones also participate in hemoglobin synthesis, both directly and indirectly. Current knowledge in these areas only scratches the surface of what is to be learned. Improved understanding of how molecular chaperones regulate erythropoietic development and hemoglobin homeostasis should identify biochemical pathways amenable to pharmacologic manipulation in a variety of red blood cell disorders including thalassemia and other anemias associated with hemoglobin instability.


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