• Min-Hui Cui, Henny H Billett, Sandra M Suzuka, Kamalakar Ambadipudi, Seetharama Archarya, Wenzhu B Mowrey, and Craig A Branch.
• Gruss Magnetic Resonance Research Center, Albert Einstein College of Medicine, New York, New York; Department of Radiology, Albert Einstein College of Medicine, New York, New York; Department of Medicine, Albert Einstein College of Medicine, New York, New York.
• Transl Res. 2022 Jun 1; 244: 75-87.

AbstractFetal hemoglobin (HbF) is known to lessen the severity of sickle cell disease (SCD), through reductions in peripheral vaso-occlusive disease and reduced risk for cerebrovascular events. However, the influence of HbF on oxygen delivery to high metabolism tissues like the brain, or its influence on cerebral perfusion, metabolism, inflammation or function have not been widely studied. We employed a Berkley mouse model (BERK) of SCD with gamma transgenes q3 expressing exclusively human α- and βS-globins with varying levels of γ globin expression to investigate the effect of HbF expression on the brain using magnetic resonance imaging (MRI), MRI diffusion tensor imaging (DTI) and spectroscopy (MRS) and hematological parameters. Hematological parameters improved with increasing γ level expression, as did markers for brain metabolism, perfusion and inflammation. Brain microstructure assessed by DTI fractional anisotropy improved, while myo-inositol levels increased, suggesting improved microstructural integrity and reduced cell loss. Our results suggest that increasing γ levels not only improves sickle peripheral disease, but also improves brain perfusion and oxygen delivery while reducing brain inflammation while protecting brain microstructural integrity.Copyright © 2022 Elsevier Inc. All rights reserved.

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