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The Primary Cilium: Emerging Role as a Key Player in Fibrosis

  • Scleroderma (J Varga, Section Editor)
  • Published:
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Abstract

Purpose of Review

The myofibroblast is the culprit in the pathogenesis of fibrosis in systemic sclerosis (SSc). Activation of morphogen signaling pathways has been shown to be critically involved in organ fibrosis. Remarkably, the cellular receptors and key molecules from these signaling pathways are localized in the primary cilium. The primary cilium is a unique cellular organelle present in virtually all cells. This article summarizes recent studies evaluating the association between primary cilia and morphogen signaling driving myofibroblast transition and subsequent fibrosis.

Recent Findings

Emerging observations implicate dysfunctional primary cilia in fibrosis in many different tissues and organs. Primary cilia seem to be necessary for the initiation of the transition and sustained activation of myofibroblasts.

Summary

We summarize recent progress in this field and propose the primary cilium as a potential mediator of fibrosis pathogenesis in SSc. Understanding the contributions of primary cilia in fibrosis may ultimately inform the development of entirely new approaches for fibrosis prevention and treatment.

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Acknowledgements

MET and JV are supported by a research award from the Scleroderma Foundation.

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Authors and Affiliations

  1. Department of Obstetrics and Gynecology, School of Medicine, Virginia Commonwealth University, 1101 E Marshall Street, Richmond, VA, 23298, USA

    Maria E. Teves, Jerome F. Strauss III & Paulene Sapao

  2. Department of Chemistry, Virginia Commonwealth University, Richmond, VA, USA

    Paulene Sapao

  3. Scleroderma Program, Division of Rheumatology, Northwestern University, 240 East Huron St., Chicago, IL, 60611, USA

    Bo Shi & John Varga

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  1. Maria E. Teves
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  2. Jerome F. Strauss III
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  3. Paulene Sapao
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Correspondence to Maria E. Teves or John Varga.

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Teves, M.E., Strauss, J.F., Sapao, P. et al. The Primary Cilium: Emerging Role as a Key Player in Fibrosis. Curr Rheumatol Rep 21, 29 (2019). https://doi.org/10.1007/s11926-019-0822-0

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