Functional correction of FA-C cells with FANCC suppresses the expression of interferon gamma-inducible genes.

Blood
S FagerlieG C Bagby

Abstract

Because hematopoietic cells derived from Fanconi anemia (FA) patients of the C-complementation group (FA-C) are hypersensitive to the inhibitory effects of interferon gamma (IFNgamma), the products of certain IFNgamma-inducible genes known to influence hematopoietic cell survival were quantified. High constitutive expression of the IFNgamma-inducible genes, IFN-stimulated gene factor 3 gamma subunit (ISGF3gamma), IFN regulatory factor-1 (IRF-1), and the cyclin-dependent kinase inhibitor p21(WAF1) was found in FANCC mutant B lymphoblasts, low-density bone marrow cells, and murine embryonic fibroblasts. Paradoxically, these cells do not activate signal transducer and activator of transcription (STAT) 1 properly. In an attempt to clarify mechanisms by which FA-C cells overexpress IFNgamma-inducible genes in the face of defective STAT1 phosphorylation, it was reasoned that decreased levels of activated STAT1 might result in reduced expression of a hematopoietic IFNgamma-responsive protein that normally modulates expression of other IFNgamma-responsive genes. Levels of the IFNgamma-inducible factor IFN consensus sequence binding protein (ICSBP), a negative trans-acting regulator of some IFNgamma-inducible genes, were quantified. ICS...Continue Reading

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Citations

May 10, 2002·BioEssays : News and Reviews in Molecular, Cellular and Developmental Biology·Shamim I AhmadSandra H Kirk
Jun 7, 2005·Biochimie·Gaëtane MacéFilippo Rosselli
Dec 26, 2001·Experimental Hematology·S FagerlieG C Bagby
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Dec 14, 2011·Hematology·Jean Soulier
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Jul 31, 2013·Blood Cells, Molecules & Diseases·Wei DuQishen Pang
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Apr 4, 2007·The Journal of Immunology : Official Journal of the American Association of Immunologists·Daniel P SejasQishen Pang

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