Figure 2.
Variably defective Ca2+ entry in platelets from patients with ORAI1W/W, ORAI1G/S or STIM1R/C mutations. Fura-2-loaded platelets in Hepes buffer with 0.1 mM EGTA were stimulated with thapsigargin (1.0 μM), and after a defined time with CaCl2 (2 mM). Platelets were analyzed from 12 healthy home controls (HC1-12), 6 healthy travel controls (C1-6), and the indicated patients/relatives (P1-6). Platelets from patient P1 were also analyzed after bone marrow (BM) transplantation. Shown are maximal increases in Ca2+. Dotted lines indicate range of store-operated Ca2+ entry (SOCE) levels (CaCl2-induced Ca2+ rise after thapsigargin) for platelets from home controls (mean ± SD, for HC1-12).