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 BEFREE AQP1 is up-regulated in SSc dermal fibroblasts and SSc endothelial cells at least partially due to autocrine TGF-β stimulation and Fli1 deficiency, respectively, possibly contributing to inflammation, vasculopathy, and tissue fibrosis by regulating tissue edema and cell migration. 30270117 2019
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma
0.300 AlteredExpression disease BEFREE Trappin-2 expression was evaluated in SSc lesional skin and cultured endothelial cells treated with FLI1 siRNA by immunohistochemistry, reverse transcription-real-time PCR and/or immunoblotting. 30520152 2019
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma
0.300 Biomarker disease BEFREE Taken together, these results indicate that cyclophosphamide improves Fli1 deficiency-dependent vascular changes by normalizing the expression of angiogenesis- and vasculogenesis-related molecules and endothelial Fli1, which may help to explain the beneficial effect of cyclophosphamide on SSc vasculopathy. 30508546 2019
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma
0.300 AlteredExpression disease BEFREE Fli1 deficiency induces endothelial adipsin expression, contributing to the onset of pulmonary arterial hypertension in systemic sclerosis. 31782787 2019
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma
0.300 Biomarker disease BEFREE PGRN overproduction due to constitutively activated c-Abl/PKC-δ/Fli1 pathway may contribute to the resistance of LSc dermal fibroblasts to the anti-fibrotic effect of TNF-α, which may be involved in maintaining their pro-fibrotic phenotype under the pro-inflammatory condition, as is the case with SSc. 30268392 2018
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma
0.300 AlteredExpression disease BEFREE Taken together, these results indicate that CXCL13 expression is upregulated by Fli1 deficiency in macrophages, potentially contributing to the development of tissue fibrosis, vasculopathy and immune activation in SSc, especially ILD and digital ulcers. 29947047 2018
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma
0.300 Biomarker disease BEFREE Friend leukemia virus integration 1 (Fli1) deficiency, a predisposing factor of systemic sclerosis (SSc), induces SSc-like phenotypes in various cell types. 29415756 2018
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
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma
0.300 Biomarker disease BEFREE We used human clinical samples and Fli1<sup>+/-</sup> mice because Fli1 deficiency induces SSc-like phenotypes in various cell types. 28528914 2017
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma
0.300 Biomarker disease BEFREE Supporting a critical role of Fli1 deficiency to induce SSc-like phenotypes, <i>CXCL6</i> mRNA expression was higher in SSc dermal fibroblasts than in normal dermal fibroblasts. 28507181 2017
CUI: C0036421
Disease: Systemic Scleroderma
Systemic Scleroderma
0.300 Biomarker disease BEFREE Collectively, these results suggest that decreased serum LIF levels may be associated with vasculopathy in SSc and that Fli1 deficiency may contribute to the inhibition of LIF-dependent biological effects on SSc endothelial cells by suppressing the expression of LIF, LIF receptor, and gp130. 29038846 2017
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 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 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