Disease: Systemic Scleroderma ×
Source: ALL
Disease Score gda Association Type Type Original DB Sentence supporting the association PMID PMID Year
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease MGD
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE In contrast to healthy skin, Fli1 protein is consistently absent from fibroblasts and significantly reduced in endothelial cells in clinically involved scleroderma skin, which correlates with enhanced collagen synthesis in systemic sclerosis skin. 12875977 2003
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease LHGDN Association between enhanced type I collagen expression and epigenetic repression of the FLI1 gene in scleroderma fibroblasts. 16802366 2006
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 PosttranslationalModification disease BEFREE Association between enhanced type I collagen expression and epigenetic repression of the FLI1 gene in scleroderma fibroblasts. 16802366 2006
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE In this article, we review the impact of Fli1 deficiency on the pathogenesis of SSc and discuss a new therapeutic strategy for SSc by targeting the transcription factor Fli1. 20663647 2010
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE Fli1 regulates genes that are involved in vessel maturation and stabilization, suggesting that reduced levels of Fli1 in SSc vasculature could contribute to the development of unstable vessels that are prone to regression. 20585340 2010
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE Endothelial Fli1 deficiency impairs vascular homeostasis: a role in scleroderma vasculopathy. 20228226 2010
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE Western blot analysis of cell lysates demonstrated that the levels of phospho-Fli-1 (Thr312) were up-regulated in SSc fibroblasts, correlating with increased levels of type I collagen and c-Abl protein. 21321929 2011
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE The expression of friend leukemia integration factor 1 (Fli1), a transcriptional repressor of collagen, was increased after treatment with ciprofloxacin only in SSc fibroblasts, and this was accompanied by a decrease in the levels of DNA methyltransferase 1 (Dnmt1). 23041765 2012
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE Decreased cathepsin V expression due to Fli1 deficiency contributes to the development of dermal fibrosis and proliferative vasculopathy in systemic sclerosis. 23287360 2013
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE These studies implicate the epigenetic downregulation of Fli1 and KLF5 as a central event triggering the pathogenic triad of SSc. 25504335 2014
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE Collectively, these results indicate that CXCL5 is a member of angiogenesis-related genes, whose expression is suppressed at least partially due to Fli1 deficiency in SSc endothelial cells. 24292093 2014
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE Transcription factor Fli-1, a deficiency of which is involved in the activation of SSc dermal fibroblasts, served as a potent repressor of the progranulin gene, and Fli-1(+/-) mice and bleomycin-treated wild-type mice exhibited up-regulated expression of progranulin in dermal fibroblasts. 26245842 2015
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE Therefore, Fli1 may serve as a predisposing factor of SSc and can be a promising therapeutic target of this incurable and devastating disease. 26055516 2015
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE Increased expression of chemerin in endothelial cells due to Fli1 deficiency may contribute to the development of digital ulcers in systemic sclerosis. 25539827 2015
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE Abnormal expression of Fli-1 is important in the etiology of autoimmune diseases such as systemic lupus erythematosus and systemic sclerosis. 24909161 2015
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE Certain cytokines or molecules, such as CD40, CD70, and Fli-1, are expressed at varying rates in SSc due to epigenetic modification and play important roles in SSc. 26043891 2015
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE The other genes (SOCS1, CTGF, THY1, CXCR4, MT1-G, FLI1, and DNMT1) were generally hypomethylated in SLE whereas they were neither hyper- nor hypo-methylated in SSc. 25986394 2015
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE This study further supports the idea that epigenetic downregulation of Fli1 is a potential predisposing factor in the pathogenesis of SSc. 25421497 2015
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE The vascular fragility of Fli-1 ECKO mice was improved by bosentan through the normalization of Fli-1 protein levels and activity in endothelial cells, which may explain, in part, the mechanism underlying the beneficial effects of endothelin receptor blockade on SSc vasculopathy. 25707716 2015
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 AlteredExpression disease BEFREE Collectively, these results suggest that endothelial CTSL up-regulation partially due to Fli1 deficiency may contribute to the development of vasculopathy, while the decrease in dermal CTSL expression is likely associated with dermal fibrosis in SSc. 26661692 2016
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE Because Krueppel-like factor (KLF)5 and Friend leukemia integration 1 transcription factor (Fli1) are the transcription factors epigenetically suppressed in SSc dermal fibroblasts, the reproduction of SSc manifestations in Klf5(+/-) ;Fli1(+/-) mice supports the canonical idea that environmental influences play a central role in the development of SSc in genetically predisposed individuals. 26782003 2016
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE Collectively, epithelial Fli1 deficiency might be involved in the systemic autoimmunity and selective organ fibrosis in SSc. 28232470 2017
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE These results indicate that Fli1 deficiency promotes migration, proliferation and cell survival, while abating tube formation of endothelial cells, suggesting that Fli1 deficiency is potentially attributable to the development of both proliferative obliterative vasculopathy (occlusion of arterioles and small arteries) and destructive vasculopathy (loss of small vessels) characteristic of SSc vasculopathy. 28370536 2017
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma 		0.300 Biomarker disease BEFREE These results indicate that glycyrrhizin ameliorates bleomycin-induced dermal fibrosis through the inhibition of fibroblast activation, T helper type 2-skewed immune polarization, M2 macrophage infiltration, and endothelial-to-mesenchymal transition and improves endothelial Fli1 deficiency-dependent vascular disintegrity, implying its potential as a disease-modifying drug for SSc. 27777101 2017