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Sustained enhancement of OCTN1 transporter expression in association with hydroxyurea induced ?-globin expression in erythroid progenitors.


ABSTRACT: The clinical benefits of hydroxyurea (HU) treatment in patients with sickle cell disease (SCD) are due largely to increased ?-globin expression. However, mechanisms that control ?-globin expression by HU in erythroid progenitors are incompletely understood. Here, we investigated the role of two HU transporters, urea transporter B (UTB) and organic cation/carnitine transporter 1 (OCTN1), in this process. Endogenous expression of both transporters peaked toward the end of erythroid differentiation. However, unlike UTB, HU-induced OCTN1 expression correlated positively with ?-globin level and was sustained throughout the period of induction. These results highlight a potential major role for OCTN1 in promoting the efficacy of HU.

SUBMITTER: Walker AL 

PROVIDER: S-EPMC5164842 | biostudies-literature | 2017 Jan

REPOSITORIES: biostudies-literature

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Sustained enhancement of OCTN1 transporter expression in association with hydroxyurea induced γ-globin expression in erythroid progenitors.

Walker Aisha L AL   Ofori-Acquah Solomon F SF  

Experimental hematology 20160908


The clinical benefits of hydroxyurea (HU) treatment in patients with sickle cell disease (SCD) are due largely to increased γ-globin expression. However, mechanisms that control γ-globin expression by HU in erythroid progenitors are incompletely understood. Here, we investigated the role of two HU transporters, urea transporter B (UTB) and organic cation/carnitine transporter 1 (OCTN1), in this process. Endogenous expression of both transporters peaked toward the end of erythroid differentiation  ...[more]

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