Properties of PK and related metabolic features in HX RBCs. (A-C) Properties of PK were assessed in HCs and patients with HX (both in PIEZO1-HX and KCNN4-HX). (A) The relative PK activity compared to the HK activity (expressed as the PK:HK ratio) was determined. (B) The PK thermostability after 60 minutes of incubation was determined. (C-E) ATP and 2,3-DPG and their ratio. (F) Given that 2,3-DPG plays a central role in hemoglobin-oxygen affinity, the p50 was measured in the 3 groups (n = 6 for PIEZO1-HX). Error bars represent IQR. ∗∗∗P < .001; ∗∗P < .01; ∗P < .05. Square symbols indicate splenectomized patients.