What question did this study set out to answer?

To investigate the differences in exported ATP and adhesivity of RBCs in individuals with sickle cell disease compared to healthy controls.

December 8, 2025BloodOpen Access

Basal and stimulated intra-RBC and exported ATP and RBC adhesivity ex vivo in children with sickle cell disease

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Analysis shows ATP export dynamics in sickle cell disease RBCs, implying potential implications for adhesion under shear stress.

To investigate the differences in exported ATP and adhesivity of RBCs in individuals with sickle cell disease compared to healthy controls.
Collected whole blood samples from patients with sickle cell disease and healthy individuals.
Incubated RBCs with AG-348 or vehicle, and performed ATP export assays under normoxic and hypoxic conditions.
Conducted adhesion assays using microfluidic channels exposed to varying shear stress levels.
Exported ATP levels increased in sickle cell RBCs after hypoxia but not with PKRA treatment.
PKRA treatment raised intra-RBC ATP in healthy controls but not in sickle cell samples.
RBC adhesion to laminin decreased following PKRA treatment under relevant shear stress conditions.

T. McMahon (2025) studied this question.

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