Sjögren (sicca = dry) Syndrome (SS)

Reference work entry
DOI: https://doi.org/10.1007/978-1-4020-6754-9_15722

Autosomal recessive autoimmune disease leading to the destruction of the salivary and lacrimal glands by the production of autoantibody against the SS-A (RoRNA) and SS-B (La Sn RNA) particles. The affected individuals have dry mouth and dry eyes (no tears). The autoantigens have been identified and purified. The Ro autoantigen appears to be encoded in human chromosome 19pter-p13.2. The La autoantigen may be involved with RNA polymerase III. The 120 kDa α-fodrin appears to be the critical autoantigen that elicits the disease. Aberrant T cells with impaired class IA phosphoinositide 3-kinase signaling can lead to organ-specific autoimmunity in mice and resemble human SS (Oak JS et al 2006 Proc Natl Acad Sci USA 103:16882).  autoimmune disease,  fodrin,  RoRNP,  PIK,  rosacea

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