Progranulin as marker for autoimmune disorders

Autoantibodies to progranulin in samples from subjects suffering from ANCA-associated and ANCA-negative primary systemic vasculitides as well as in other autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis have been identified. In addition, also progranulin with a specific posttranslational modification and aberrant forms of progranulin in these autoimmune patientshas been found.

A neutralizing effect of anti-progranulin autoantibodies on progranulin plasma levels, which is of particular interest in the light of progranulin’s function as a potent direct inhibitor of TNFR-1&2 has been demonstrated as well as the fact, that the presence of anti-progranulin autoantibodies is significantly associated with active disease.

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