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. Author manuscript; available in PMC: 2020 Mar 1.
Published in final edited form as: Clin Immunol. 2019 Jan 31;200:66–70. doi: 10.1016/j.clim.2019.01.009

Figure 1. Foreign vs. self: prediction of epitopes likely to generate an inflammatory response in IOPD ERT recipients.

Figure 1.

CRIM negative patients receiving ERT are unlikely to tolerate any T cell epitopes contained within rhGAA and restricted by their HLA due to incomplete thymic education (left), resulting in all rhGAA epitopes being perceived as “foreign”. CRIM positive patients, who may express residual protein, may tolerate those “self” rhGAA epitopes present in their nGAA, but are likely to generate inflammatory responses to “foreign” rhGAA T cell epitopes present in regions corresponding to truncated or mutated portions of their specific nGAA sequences (right).