Journal
BLOOD CELLS MOLECULES AND DISEASES
Volume 52, Issue 1, Pages 2-11Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.bcmd.2013.06.005
Keywords
Fanconi anemia; DNA repair; TNF-alpha; Hematopoietic failure inflammation; Leukemia
Categories
Funding
- NIH [R01 HL076712, R01 CA157537]
- Leukemia and Lymphoma Scholar award
- NIH
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Tumor necrosis factor-alpha (TNF-alpha) is a major pro-inflammatory cytokine involved in systemic inflammation and the acute phase reaction. Dysregulation of TNF production has been implicated in a variety of human diseases including Fanconi anemia (FA). FA is a genomic instability syndrome characterized by progressive bone marrow failure and cancer susceptibility. The patients with FA are often found overproducing TNF-alpha, which may directly affect hematopoietic stem cell (HSC) function by impairing HSC survival, homing and proliferation, or indirectly change the bone marrow microenvironment critical for HSC homeostasis and function, therefore contributing to disease progression in FA. In this brief review, we discuss the link between TNF-a signaling and FA pathway with emphasis on the implication of inflammation in the pathophysiology and abnormal hematopoiesis in FA. (C) 2013 Elsevier Inc. All rights reserved.
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