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Condition: Scleroderma

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Total 44 results found since Jan 2013.

ATRA ameliorates fibrosis by suppressing the pro-fibrotic molecule Fra2/AP-1 in systemic sclerosis
In this study, the pro-fibrotic effect of Fra2 in SSc is confirmed. SSc dermal fibroblasts and bleomycin-induced fibrotic tissues of SSc animals exhibit increased levels of Fra2. Inhibition of Fra2 expression in SSc dermal fibroblasts with Fra2 siRNA markedly decreased collagen I expression. ATRA reduced the expressions of Fra2, collagen I, and α-smooth muscle actin(α-SMA) in SSc dermal fibroblasts and bleomycin-induced fibrotic tissues of SSc mice. In addition, chromatin immunoprecipitation and dual-luciferase assays demonstrated that retinoic acid receptor RARα binds to the FRA2 promoter and modulates its transcriptio...
Source: International Immunopharmacology - June 18, 2023 Category: Allergy & Immunology Authors: Zixin Pi Jiani Liu Yangfan Xiao Xinglan He Ruixuan Zhu Rui Tang Xiangning Qiu Yi Zhan Zhuotong Zeng Yaqian Shi Rong Xiao Source Type: research

Increased Expression of LMCD1 in Scleroderma ‐Associated Interstitial Lung Disease is Critical for Profibrotic Characteristics of Lung Myofibroblasts
ConclusionOur study identifies LMCD1 as a profibrotic molecule contributing to the activation of myofibroblasts and the persistent fibroproliferation observed in SSc-ILD. Thus, LMCD1 may be a potential novel therapeutic target for patients with SSc-ILD.This article is protected by copyright. All rights reserved.
Source: Arthritis and Rheumatology - September 14, 2022 Category: Rheumatology Authors: Galina S. Bogatkevich, Ilia Atanelishvili, Andrew M. Bogatkevich, Richard M. Silver Tags: Full Length Source Type: research

NF- кB c-Rel modulates pre-fibrotic changes in human fibroblasts
Arch Dermatol Res. 2021 Dec 9. doi: 10.1007/s00403-021-02310-2. Online ahead of print.ABSTRACTSkin fibrosis is one central hallmark of the heterogeneous autoimmune disease systemic sclerosis. So far, there are hardly any standardized and effective treatment options. Pathogenic mechanisms underlying fibrosis comprise excessive and uncontrolled myofibroblast differentiation, increased extracellular matrix protein (ECM) synthesis and an intensification of the forces exerted by the cytoskeleton. A deeper understanding of fibroblast transformation could help to prevent or reverse fibrosis by specifically interfering with abnorm...
Source: Cell Research - December 10, 2021 Category: Cytology Authors: Lara Carolina Micus Franziska Susanne Trautschold-Krause Anna Lena Jelit Michael Peter Sch ön Verena Natalie Lorenz Source Type: research

Nimbolide attenuated the inflammation in the liver of autoimmune hepatitis's mice through regulation of HDAC3
Toxicol Appl Pharmacol. 2021 Nov 12:115795. doi: 10.1016/j.taap.2021.115795. Online ahead of print.ABSTRACTA chronic liver disease named autoimmune hepatitis (AIH) will carry elevated levels of inflammatory cytokines, but there is currently no effective treatment to cure it. Histone deacetylase 3 (HDAC3) takes an important position in regulating the expression of inflammatory genes. Nimbolide (NIB) is a limonoid extracted from the neem tree (Azadirachta indica) that has been found to be effective against many diseases, including cancer, scleroderma, and acute respiratory distress syndrome. Here, we investigated the protect...
Source: Toxicology and Applied Pharmacology - November 15, 2021 Category: Toxicology Authors: Dingchao Xia Dazhi Chen Tingchen Cai Lujian Zhu Yanhan Lin Sijie Yu Kailu Zhu Xiaodong Wang Lanman Xu Yongping Chen Source Type: research

Selective deletion of Connective Tissue Growth Factor attenuates experimentally -induced pulmonary fibrosis and pulmonary arterial hypertension
In this study, genetic manipulation of the CCN2 gene was employed to investigate the role of CCN2 expression in vitro and in experimentally-induced models of pulmonary fibrosis and pulmonary arterial hypertension (PAH). Knocking down CCN2 using siRNA, reduced expression of pro-fibrotic markers (fibronectin p < 0.01, collagen type I p < 0.05, α-SMA p < 0.0001, TIMP-1 p < 0.05 and IL-6 p < 0.05) in TGF-β-treated lung fibroblasts derived from systemic sclerosis patients. In vivo studies were performed in mice using a conditional gene deletion strategy targeting CCN2 in a fibroblast-specific and time-dependent...
Source: The International Journal of Biochemistry and Cell Biology - March 4, 2021 Category: Biochemistry Authors: Angela Y Y Tam Amy L Horwell Sarah L Trinder Korsa Khan Shiwen Xu Voon Ong Christopher P Denton Jill T Norman Alan M Holmes George Bou-Gharios David J Abraham Source Type: research

GSE162966 IL11 is elevated in systemic sclerosis and IL11-dependent ERK signaling is needed for the profibrotic activity of TGF β in dermal fibroblasts
Conclusions: These data reveal an important contribution of IL11-related ERK activity for TGF β-stimulated fibrosis phenotypes in HDFs and suggest IL11 as a potential therapeutic target in SSc.
Source: GEO: Gene Expression Omnibus - February 22, 2021 Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research

Expression of pituitary tumor-transforming gene-1 and its pathogenic role in systemic sclerosis.
CONCLUSIONS: PTTG1 is highly expressed in skin tissues of patients with SSc, and PTTG1 knockdown can reduce the activity of the dermal fibroblasts, suggesting a close correlation of PTTG1 with fibrosis in SSc. PMID: 33243736 [PubMed - in process]
Source: Journal of Southern Medical University - November 29, 2020 Category: Universities & Medical Training Authors: Yang A, Huang Y, Zhang Y, Yang K, Wang J, Liu Q Tags: Nan Fang Yi Ke Da Xue Xue Bao Source Type: research

Fli1 Downregulation in Scleroderma Myeloid Cells Has Profibrotic and Proinflammatory Effects
Scleroderma (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation, vasculopathy, and fibrosis. We have previously demonstrated that low Fli1 expression in SSc fibroblasts and endothelial cells plays an important role in SSc pathogenesis. Cells of myeloid and lymphoid origin also express Fli1 and are dysregulated in patients with SSc, playing key roles in disease pathogenesis. However, the role for immune Fli1 in SSc is not yet clear. Our aim was to elucidate whether Fli1 contributes to the immune dysregulation seen in SSc. Comparison of the expression of Fli1 in monocytes, B- and T-cell fra...
Source: Frontiers in Immunology - May 18, 2020 Category: Allergy & Immunology Source Type: research

CXCL4 triggers monocytes and macrophages to produce PDGF-BB, culminating in fibroblast activation: Implications for systemic sclerosis.
CONCLUSION: Our findings demonstrate that CXCL4 can drive fibroblast activation indirectly via PDGF-BB production by myeloid cells. Hence, targeting PDGF-BB or CXCL4-induced PDGF-BB release could be clinically beneficial for patients with SSc. PMID: 32284212 [PubMed - as supplied by publisher]
Source: Journal of Autoimmunity - April 9, 2020 Category: Allergy & Immunology Authors: van der Kroef M, Carvalheiro T, Rossato M, de Wit F, Cossu M, Chouri E, Wichers CGK, Bekker CPJ, Beretta L, Vazirpanah N, Trombetta E, Radstake TRDJ, Angiolilli C Tags: J Autoimmun Source Type: research