학술논문
Effects of pyruvate kinase activators on red blood cell properties in sickle cell disease.
Document Type
Article
Author
Source
Subject
*SICKLE cell anemia
*ERYTHROCYTES
*PYRUVATE kinase
*HYDROPEROXIDES
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Language
ISSN
0007-1048
Abstract
For the experiments performed without cumene hydroperoxide, 23 patients were included for haemoglobin-oxygen affinity and RBC properties analyses (26 ± 13 years; HbF = 12.5 ± 7.9%; HbS = 82.0 ± 10.1%, Hb = 8.8 ± 1.3 g/dL, reticulocyte count = 230 ± 116 10 SP 9 sp /L, 7 under hydroxyurea) and 17 others were included for the RBC senescence markers measurements (23 ± 11 years; HbF = 15.0 ± 12.0%; HbS = 79.0 ± 13.0%, Hb = 9.1 ± 1.8 g/dL, 195 ± 109 10 SP 9 sp /L, 13 under hydroxyurea). For the second set of experiments, RBCs were incubated with PKAs for 4 h, and then, cumene hydroperoxide (0.4 mM) was added to the RBCs suspension for 15 min (37°C) to mimic a situation of increased oxidative stress. *p < 0.05; **p < 0.01;; ****p < 0.0001; ns, no significant difference. gl Our results show that AG946, like mitapivat, increases the haemoglobin-oxygen affinity and reduces RBC sickling in patients with SCD ex-vivo. [Extracted from the article]