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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |
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 |