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The Epigenetic Regulation of Scleroderma and Its Clinical Application

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Book cover Epigenetics in Allergy and Autoimmunity

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 1253))

Abstract

Scleroderma (systemic sclerosis; SSc) is a complex and highly heterogeneous multisystem rheumatic disease characterized by vascular abnormality, immunologic derangement, and excessive deposition of extracellular matrix (ECM) proteins. To date, the etiology of this life-threatening disorder remains not fully clear. More and more studies show epigenetic modifications play a vital role. The aberrant epigenetic status of certain molecules such as Fli-1, BMPRII, NRP1, CD70, CD40L, CD11A, FOXP3, KLF5, DKK1, SFRP1, and so on contributes to the pathogenesis of progressive vasculopathy, autoimmune dysfunction, and tissue fibrosis in SSc. Meanwhile, numerous miRNAs including miR-21, miR-29a, miR-196a, miR-202-3p, miR-150, miR-let-7a, and others are involved in the process. In addition, the abnormal epigenetic biomarker levels of CD11a, Foxp3, HDAC2, miR-30b, miR-142-3p, miR-150, miR-5196 in SSc are closely correlated with disease severity. In this chapter, we not only review new advancements on the epigenetic mechanisms involved in the pathogenesis of SSc and potential epigenetic biomarkers, but also discuss the therapeutic potential of epigenetic targeting therapeutics such as DNA methylation inhibitors, histone acetylase inhibitors, and miRNA replacement.

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Authors and Affiliations

  1. Department of Dermatology, Hunan Children’s Hospital, Changsha, China

    Yangyang Luo

  2. Department of Dermatology, The Second Xiangya Hospital, Central South University, Changsha, China

    Rong Xiao

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  1. Yangyang Luo
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  2. Rong Xiao
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Correspondence to Rong Xiao .

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Editors and Affiliations

  1. Division of Pediatric Immunology and Allergy, Joe DiMaggio Children’s Hospital, Hollywood, FL, USA

    Christopher Chang

  2. Department of Dermatology, Hunan Key Laboratory of Medical Epigenomics, Second Xiangya Hospital, Central South University, Changsha, Hunan, China

    Qianjin Lu

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Luo, Y., Xiao, R. (2020). The Epigenetic Regulation of Scleroderma and Its Clinical Application. In: Chang, C., Lu, Q. (eds) Epigenetics in Allergy and Autoimmunity. Advances in Experimental Medicine and Biology, vol 1253. Springer, Singapore. https://doi.org/10.1007/978-981-15-3449-2_13

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