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

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.

• Authors: Dana P Ascherman
• Journal: Current rheumatology reports (Curr Rheumatol Rep) Vol. 17 Issue 9 Pg. 56 (Sep 2015) ISSN: 1534-6307 [Electronic] United States
• PMID: 26210509 (Publication Type: Journal Article, Review)

Chemical References

• Antibodies, Antinuclear
• Jo-1 antibody
• Histidine-tRNA Ligase

Topics

• Adaptive Immunity
• Antibodies, Antinuclear (blood)
• Autoimmune Diseases (immunology)
• Histidine-tRNA Ligase (immunology)
• Humans
• Immunity, Innate
• Lung Diseases, Interstitial (epidemiology, immunology)
• Myositis (epidemiology, immunology)
• Syndrome