Recent Advances in "sickle and Niche" Research - Tribute to Dr. Paul S Frenette
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In this retrospective, we review the two research topics that formed the basis of the outstanding career of Dr. Paul S. Frenette. In the first part, we focus on sickle cell disease (SCD). The defining feature of SCD is polymerization of the deoxygenated mutant hemoglobin, which leads to a vicious cycle of hemolysis and vaso-occlusion. We survey important discoveries in SCD pathophysiology that have led to recent advances in treatment of SCD. The second part focuses on the hematopoietic stem cell (HSC) niche, the complex microenvironment within the bone marrow that controls HSC function and homeostasis. We detail the cells that constitute this niche, and the factors that these cells use to exert control over hematopoiesis. Here, we trace the scientific paths of Dr. Frenette, highlight key aspects of his research, and identify his most important scientific contributions in both fields.
Su S, Bao W, Liu Y, Shi P, Manwani D, Murakhovskaya I Blood. 2024; 145(3):334-347.
PMID: 39656114 PMC: 11775509. DOI: 10.1182/blood.2024025175.
Maddah R, Etemad S, Amiri B, Ghaderi H, Zarei H, Faghihkhorasani F Iran J Public Health. 2024; 53(6):1404-1415.
PMID: 39430157 PMC: 11488559.
Bonora M, Morganti C, van Gastel N, Ito K, Calura E, Zanolla I Cell Stem Cell. 2024; 31(3):359-377.e10.
PMID: 38458178 PMC: 10957094. DOI: 10.1016/j.stem.2024.02.004.
Quarato E, Salama N, Li A, Smith C, Zhang J, Kawano Y Cell Death Dis. 2023; 14(7):428.
PMID: 37452070 PMC: 10349065. DOI: 10.1038/s41419-023-05931-9.
A mechanosensitive vascular niche for hematopoiesis.
Tian Y, Morin-Poulard I, Liu X, Vanzo N, Crozatier M Proc Natl Acad Sci U S A. 2023; 120(18):e2217862120.
PMID: 37094122 PMC: 10160988. DOI: 10.1073/pnas.2217862120.