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. 2012 May 24;122(6):2141–2152. doi: 10.1172/JCI61788

Figure 6. ADA HSC-GT corrects central B cell tolerance in ADA-SCID patients.

Figure 6

(A) Antibodies expressed by new emigrant/transitional B cells from ADA-SCID patients were tested by ELISA for polyreactivity against dsDNA, insulin, and LPS. Dotted lines show ED38-positive control. Horizontal lines show cutoff OD405 for positive reactivity. The frequencies of polyreactive and nonpolyreactive clones are summarized in the pie charts, with the number of antibodies tested indicated in the center. (B) Polyreactive new emigrant/transitional B cell frequency decreased in ADA-SCID patients after HSC-GT, but remained significantly elevated compared with that in HDs. (C) ADA HSC-GT completely restored the central removal of ANA-expressing developing B cells. ANA-expressing new emigrant/transitional B cell frequency in ADA-SCID patients after HSC-GT was significantly decreased compared with pretherapy frequencies, comparable to those in HDs. (B and C) Each symbol represents an individual, horizontal bars denote means, and dashed lines show the average frequency in HDs. *P ≤ 0.05. (D) Antibodies expressed from ADA-SCID new emigrant/transitional B cells showed various cytoplasmic staining patterns and were devoid of nuclear reactivity. 2 examples for anticytoplasmic new emigrant B cells are shown for each patient. Original magnification, ×40.