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Role of Jo-1 in the Immunopathogenesis of the Anti-synthetase Syndrome

  • Inflammatory Muscle Disease (Myositis) (R Aggarwal, Section Editor)
  • Published:
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Abstract

Histidyl-tRNA synthetase (HRS = Jo-1) represents a key autoantibody target in the anti-synthetase syndrome that is marked by myositis as well as extra-muscular organ complications including interstitial lung disease (ILD). Over the last 25 years, a wealth of clinical, epidemiological, genetic, and experimental data have collectively supported a role for Jo-1 in mediating deleterious cell-mediated, adaptive immune responses contributing to the disease phenotype of the anti-synthetase syndrome. Complementing these studies, more recent work suggests that unique, non-enzymatic functional properties of Jo-1 also endow this antigen with the capacity to activate components of the innate immune system, particularly cell surface as well as endosomal Toll-like receptors and their downstream signaling pathways. Combining these facets of Jo-1-mediated immunity now supports a more integrated model of disease pathogenesis that should lead to improved therapeutic targeting in the anti-synthetase syndrome and related subsets of idiopathic inflammatory myopathy.

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The author declares that she has no competing interests.

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  1. Division of Rheumatology, Department of Medicine, Miller School of Medicine, University of Miami, RMSB, 7152, 1600 NW 10th Avenue, Miami, FL, 33136, USA

    Dana P. Ascherman

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  1. Dana P. Ascherman
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Correspondence to Dana P. Ascherman.

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This article is part of the Topical Collection on Inflammatory Muscle Disease

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Ascherman, D.P. Role of Jo-1 in the Immunopathogenesis of the Anti-synthetase Syndrome. Curr Rheumatol Rep 17, 56 (2015). https://doi.org/10.1007/s11926-015-0532-1

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