Background: Potential target autoantigens in the demyelinating form of Guillain-Barré Syndrome (GBS) include the myelin proteins PMP22, P0 and P2.
Methods: We investigated immunoreactivity to P0, P2 and PMP22 proteins in 37 GBS patients and 32 healthy controls.
Results: Antibodies to PMP22 or P0 peptides were detected at presentation in only 5 out of 37 patients. In ELISPOT assays, blood mononuclear cells from 15 out of 24 GBS patients but none of the control subjects produced interleukin-10 (IL-10) in response to peptides from proteins P0, P2 or PMP22 (p = 0.0003). The cells from only two patients produced interferonγ (IFNγ).The cells from 11 GBS patients had increased IL-10 responses to peptides representing sequences from the extracellular domains of PMP22 before intravenous immunoglobulin (IVIg) treatment (p = 0.006). The cells from 11 GBS patients, including seven who responded to the extracellular domains of PMP22, had increased IL-10 responses to the intracellular domain of P0 before (p = 0.005) and those from 9 patients after they had been treated with IVIg (p = 0.01).
Conclusions: Antibodies to P0 and PMP22 protein peptides do occur in GBS but are uncommon. Circulating mononuclear cell IFNγ responses to P0 and PMP22 myelin proteins are rare but IL-10 responses occur significantly more often than in normal subjects. They might be part of a harmful pathogenetic process or represent a regulatory response.
- Guillain-Barré syndrome
- T cell