Review
Biochemistry & Molecular Biology
Yuko Ishida, Yumi Kuninaka, Naofumi Mukaida, Toshikazu Kondo
Summary: Fibrosis and structural remodeling of lung tissue can seriously impact lung function and have fatal consequences. The causes of pulmonary fibrosis (PF) vary, including allergens, chemicals, radiation, and environmental particles. The cause of idiopathic PF (IPF), one of the most common forms, is still unknown. The murine bleomycin (BLM) model has been extensively studied to understand the mechanisms of PF.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Rheumatology
Benjamin E. Decato, Ron Ammar, Lauren Reinke-Breen, John R. Thompson, Anthony Azzara
Summary: This study explored the molecular basis for SSc pathogenesis in a mouse model of scleroderma, identifying changes in gene expression related to anti-fibrotic therapy and implementing a machine learning algorithm for predicting lung function using transcriptome data. The findings provide insights into SSc pathogenesis and intervention pathways, offering a rich dataset for future fibrotic disease research.
Article
Biochemistry & Molecular Biology
Theodoros-Ioannis Papadimitriou, Arjan van Caam, Peter M. van der Kraan, Rogier M. Thurlings
Summary: This review examines the relationship between inflammation and fibrosis in systemic sclerosis, as well as the efficacy of current and novel treatment options in reducing fibrosis.
Article
Cell Biology
Jong Seong Roh, Hoim Jeong, Beomgu Lee, Byung Wook Song, Seung Jin Han, Dong Hyun Sohn, Seung-Geun Lee
Summary: The study revealed that mirodenafil has the potential to alleviate skin fibrosis caused by SSc by downregulating the TGF-β/Smad signaling pathway.
ANIMAL CELLS AND SYSTEMS
(2021)
Article
Biochemistry & Molecular Biology
Jingjing Huang, Hydia Puente, Nancy E. E. Warening, Minghua Wu, Maureen D. D. Mayes, Harry Karmouty-Quintana, Shervin Assassi, Tingting W. W. Mills
Summary: This study found that phosphorylation of STAT6 increased in fibrotic skin samples from SSc patients and bleomycin-injected mice. Knockout of Stat6 in mice suppressed fibrotic cytokines expression and reduced collagen and fibronectin production. STAT6 inhibition also attenuated skin fibrosis in mice. Co-culture experiments further demonstrated the role of STAT6 in cytokine and fibrotic marker expression in skin epithelial cells and fibroblasts.
Article
Chemistry, Medicinal
Gyu-Tae Park, Jung-Won Yoon, Sang-Bin Yoo, Young-Chul Song, Parkyong Song, Hyoung-Kyu Kim, Jin Han, Sung-Jin Bae, Ki-Tae Ha, Natalia P. Mishchenko, Sergey A. Fedoreyev, Valentin A. Stonik, Moon-Bum Kim, Jae-Ho Kim
Summary: The study demonstrates that Echinochrome A treatment can alleviate bleomycin-induced scleroderma by normalizing dermal thickness and suppressing collagen deposition, reducing the number of activated myofibroblasts and macrophages in the affected skin, and decreasing serum levels of inflammatory cytokines. EchA shows promise for treating scleroderma by exerting anti-fibrosis and anti-inflammatory effects.
Article
Biochemistry & Molecular Biology
Hee Jin Park, Ok-Yi Jeong, Sung Hak Chun, Yun Hong Cheon, Mingyo Kim, Suhee Kim, Sang-Il Lee
Summary: The study found that butyrate has potential therapeutic effects in a mouse model of SSc and human dermal fibroblasts, by modulating intestinal microbiota, controlling macrophage differentiation, and inhibiting proinflammatory gene expression, thus improving fibrosis.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Immunology
Dominique Farge, Severine Loisel, Pauline Lansiaux, Karin Tarte
Summary: Systemic sclerosis (SSc) is a rare chronic autoimmune disease characterized by vasculopathy, dysregulation of immune responses, and progressive fibrosis. Mesenchymal stromal cells (MSC) have shown promise as a potential stem cell therapy for SSc, with studies demonstrating proangiogenic, immuno-suppressive, and anti-fibrotic properties. Preclinical studies in animal models and early clinical trials in humans have reported positive results in reducing fibrosis in SSc patients.
AUTOIMMUNITY REVIEWS
(2021)
Article
Dermatology
Zhixing Jiang, Chen Chen, Sen Yang, Hang He, Xiaoxia Zhu, Minrui Liang
Summary: The chemokine CXCL4 is associated with anti-angiogenic properties and plays a role in systemic sclerosis (SSc) related pulmonary arterial hypertension (PAH). Elevated levels of circulating CXCL4 were found in SSc patients and correlated with peripheral vasculopathy, with potential effects on endothelial cell dysfunction and angiogenesis. The study suggests that CXCL4 may contribute to peripheral vasculopathy in SSc through downregulating Fli-1 via c-Abl signaling in endothelial cells.
JOURNAL OF DERMATOLOGICAL SCIENCE
(2021)
Review
Immunology
Wei Jin, Yan Zheng, Ping Zhu
Summary: Systemic sclerosis is an autoimmune disease with a poor prognosis. T cell abnormalities play a crucial role in its pathogenesis and treatment. Understanding the role of T cells may lead to more targeted treatment options for systemic sclerosis patients.
AUTOIMMUNITY REVIEWS
(2022)
Review
Rheumatology
Jakob Hoeppner, Cosimo Bruni, Oliver Distler, Simon C. Robson, Gerd R. Burmester, Elise Siegert, Jorg H. W. Distler
Summary: SSc is a chronic autoimmune rheumatic disease with histopathological hallmarks including vasculopathy, fibrosis, and autoimmune phenomena. Purinergic signalling pathway may play a role in the pathophysiology of SSc, exacerbating vasculopathy and immune dysregulation, while also promoting organ and tissue fibrosis. Targeting purinergic signalling could offer new therapeutic options for SSc.
Article
Rheumatology
Dianyu Cao, Jina Zheng, Zheng Li, Yong Yu, Zengrui Chen, Qiang Wang
Summary: The study demonstrates that ACSL4 induces inflammatory macrophage ferroptosis, exacerbating fibrosis progression in SSc. ACSL4 and its regulators calpains may serve as potential therapeutic targets for SSc.
ARTHRITIS RESEARCH & THERAPY
(2023)
Article
Dermatology
Wah Wah Aung, Chenyang Wang, Jia Xibei, Motoki Horii, Kie Mizumaki, Miyu Kano, Ai Okamura, Tadahiro Kobayashi, Takashi Matsushita
Summary: Tofacitinib showed significant improvement in fibrosis in a mouse model of systemic sclerosis by suppressing immune responses of T and B cells, proinflammatory cells, and macrophages, and reducing the expression of fibrotic cytokines and proteins.
JOURNAL OF DERMATOLOGICAL SCIENCE
(2021)
Article
Biochemistry & Molecular Biology
Hana Storkanova, Lenka Storkanova, Adela Navratilova, Viktor Becvar, Hana Hulejova, Sabina Oreska, Barbora Hermankova, Maja Spiritovic, Radim Becvar, Karel Pavelka, Jiri Vencovsky, Joerg H. W. Distler, Ladislav Senolt, Michal Tomcik
Summary: This study demonstrates the crucial role of Hsp90 in the pathophysiology of SSc and identifies it as a potential target for fibrosis treatment. Treatment with the Hsp90 inhibitor 17-DMAG effectively prevents and reverses dermal fibrosis, showing significant anti-fibrotic and anti-inflammatory properties.
Article
Biochemistry & Molecular Biology
Yosuke Kanno, En Shu, Hirofumi Niwa, Mariko Seishima, Kei-ichi Ozaki
Summary: The study found that miR-30c can reduce the expression of α2AP, alleviate fibrotic changes and vascular dysfunction in the skin of SSc model mice, and also suppress pulmonary fibrosis progression.
MOLECULAR BIOLOGY REPORTS
(2021)