Sticking together: Polymerization of sickle hemoglobin drives the multiscale pathophysiology of sickle cell disease.

IF 2.9 Q2 BIOPHYSICS
Biophysics reviews Pub Date : 2025-03-20 eCollection Date: 2025-03-01 DOI:10.1063/5.0238698
Dillon C Williams, Hannah M Szafraniec, David K Wood
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引用次数: 0

Abstract

Sickle cell disease is a hereditary disorder in which the pathophysiology is driven by the aggregation of a mutant (sickle) hemoglobin (HbS). The self-assembly of deoxygenated sickle hemoglobin molecules into ordered fiber structures has consequences extending to the cellular and rheological levels, stiffening red blood cells and inducing pathological flow behavior. This review explores the current understanding of the molecular processes involved in the polymerization of hemoglobin in sickle cell disease and how the molecular phase transition creates quantifiable changes at the cellular and rheological scale, as well as, identifying knowledge gaps in the field that would improve our understanding of the disease and further improve treatment and management of the disease.

粘在一起:镰状血红蛋白的聚合驱动镰状细胞病的多尺度病理生理。
镰状细胞病是一种遗传性疾病,其病理生理是由突变(镰状)血红蛋白(HbS)聚集驱动的。脱氧的镰状血红蛋白分子自组装成有序的纤维结构,其后果延伸到细胞和流变学水平,使红细胞硬化并诱导病理流动行为。这篇综述探讨了目前对镰状细胞病中血红蛋白聚合的分子过程的理解,以及分子相变如何在细胞和流变尺度上产生可量化的变化,以及确定该领域的知识空白,这将提高我们对疾病的理解,并进一步改善疾病的治疗和管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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CiteScore
3.60
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0.00%
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