PMID- 23890415 OWN - NLM STAT- MEDLINE DCOM- 20140714 LR - 20211021 IS - 1096-0961 (Electronic) IS - 1079-9796 (Print) IS - 1079-9796 (Linking) VI - 52 IP - 1 DP - 2014 Jan TI - TNF-alpha signaling in Fanconi anemia. PG - 2-11 LID - S1079-9796(13)00151-4 [pii] LID - 10.1016/j.bcmd.2013.06.005 [doi] AB - 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-alpha signaling and FA pathway with emphasis on the implication of inflammation in the pathophysiology and abnormal hematopoiesis in FA. CI - (c) 2013. FAU - Du, Wei AU - Du W AD - Division of Experimental Hematology and Cancer Biology, University of Cincinnati College of Medicine, Cincinnati, OH 45229, USA. Electronic address: wei.du@cchmc.org. FAU - Erden, Ozlem AU - Erden O FAU - Pang, Qishen AU - Pang Q LA - eng GR - P30 DK090971/DK/NIDDK NIH HHS/United States GR - R01 CA157537/CA/NCI NIH HHS/United States GR - R01 HL076712/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20130724 PL - United States TA - Blood Cells Mol Dis JT - Blood cells, molecules & diseases JID - 9509932 RN - 0 (Fanconi Anemia Complementation Group Proteins) RN - 0 (Tumor Necrosis Factor-alpha) SB - IM MH - Apoptosis MH - Bone Marrow/immunology/*pathology MH - Cell Proliferation MH - Cell Survival MH - Cell Transformation, Neoplastic/immunology/pathology MH - Fanconi Anemia/*genetics/immunology/pathology MH - Fanconi Anemia Complementation Group Proteins/*genetics/immunology MH - Gene Expression Regulation MH - Genomic Instability MH - Hematopoietic Stem Cells/immunology/*pathology MH - Humans MH - Inflammation/genetics/immunology/pathology MH - Signal Transduction/*genetics/immunology MH - Tumor Necrosis Factor-alpha/*genetics/immunology PMC - PMC3851925 MID - NIHMS509990 OTO - NOTNLM OT - DNA repair OT - Fanconi anemia OT - Hematopoietic failure inflammation OT - Leukemia OT - TNF-alpha EDAT- 2013/07/31 06:00 MHDA- 2014/07/16 06:00 PMCR- 2015/01/01 CRDT- 2013/07/30 06:00 PHST- 2013/06/18 00:00 [received] PHST- 2013/06/20 00:00 [revised] PHST- 2013/06/20 00:00 [accepted] PHST- 2013/07/30 06:00 [entrez] PHST- 2013/07/31 06:00 [pubmed] PHST- 2014/07/16 06:00 [medline] PHST- 2015/01/01 00:00 [pmc-release] AID - S1079-9796(13)00151-4 [pii] AID - 10.1016/j.bcmd.2013.06.005 [doi] PST - ppublish SO - Blood Cells Mol Dis. 2014 Jan;52(1):2-11. doi: 10.1016/j.bcmd.2013.06.005. Epub 2013 Jul 24.