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The Ro 60 kDa autoantigen: insights into cellular function and role in autoimmunity

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Abstract

An RNA-binding protein, the Ro 60 kDa autoantigen, is a major target of the immune response in patients suffering from two systemic rheumatic diseases, systemic lupus erythematosus and Sjogren’s syndrome. In lupus patients, anti-Ro antibodies are associated with photosensitive skin lesions and with neonatal lupus, a syndrome in which mothers with anti-Ro antibodies give birth to children with photosensitive skin lesions and a cardiac conduction defect, third degree heart block. In vertebrate cells, the Ro protein binds small RNAs of unknown function known as Y RNAs. Although the cellular function of Ro has long been mysterious, recent studies have implicated Ro in two distinct processes: small RNA quality control and the enhancement of cell survival following exposure to ultraviolet irradiation. Most interestingly, mice lacking the Ro protein develop an autoimmune syndrome that shares some features with systemic lupus erythematosus in patients, suggesting that the normal function of Ro may be important for the prevention of this autoimmune disease. In this review, we summarize recent progress towards understanding the role of the Ro 60 kDa protein and discuss whether the cellular function of Ro could be related to certain manifestations of lupus in patients.

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Abbreviations

RNP :

Ribonucleoprotein

RRM :

RNA recognition motif

SCLE :

Subacute cutaneous lupus erythematosus

SLE :

Systemic lupus erythematosus

snRNP :

Small nuclear ribonucleoprotein

SS :

Sjogren’s syndrome

UV :

Ultraviolet

VWFA :

von Willebrand factor A

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Acknowledgements

We thank Carl Hashimoto, Karin Reinisch, and Elisabetta Ullu for comments on the manuscript. S.L.W. is an Associate Investigator of the Howard Hughes Medical Institute.

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  1. Departments of Cell Biology and Molecular Biophysics and Biochemistry, Howard Hughes Medical Institute, Yale University School of Medicine, 295 Congress Avenue, New Haven, CT 06536, USA

    Xinguo Chen & Sandra L. Wolin

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Correspondence to Sandra L. Wolin.

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Chen, X., Wolin, S.L. The Ro 60 kDa autoantigen: insights into cellular function and role in autoimmunity. J Mol Med 82, 232–239 (2004). https://doi.org/10.1007/s00109-004-0529-0

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