Reactive Species in Sickle Cell Disease
Abstract
A bstract: The red cell is a relatively abundant locus of both free radical generation and reaction. Erythrocytes have a high content of unsaturated membrane lipids, a rich oxygen supply and are densely packed with redox‐active hemoglobin residues. In response, red cells have a highly evolved and well‐integrated network of oxidant defense mechanisms that lend an ability to withstand oxidative stress. In the case of congenital hemoglobin mutations that underlie sickle cell disease, they become very susceptible to free radical‐mediated injury by virtue of enhanced endogenous rates of production of reactive species and impairment of tissue free radical defense mechanisms. In sickle cell disease, a combination of these susceptibility factors are hypothesized to lead to an overall impairment of vascular function, in large part due to loss of “bioactive” nitric oxide via the free radical‐mediated consumption of this vasoactive molecule.
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Sickle cells produce greater amounts of O2•−, H2O2, and hydroxyl radical •OH compared with healthy RBCs.
“It has been reported that sickle cells produce greater amounts of O2•−, H2O2, and hydroxyl radical •OH compared with healthy RBCs (8, 80, 100).”
From Red Blood Cell Function and Dysfunction: Redox Regulation, Nitric Oxide Metabolism, Anemia (2017) · cites this paper as [8]
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- Authors
- ASLAN, M., THORNLEY‐BROWN, D., & FREEMAN, B. A.
- Affiliations
- University of Alabama at Birmingham (US)
- ORCID
- ASLAN, MUTAY
- Venue
- Annals of the New York Academy of Sciences
- Publisher
- Wiley
- Published
- 2000-01
- Volume
- 899
- Issue
- 1
- Pages
- 375-391
- ISSN
- 0077-8923 (ISSN-L)
- 1749-6632
- OpenAlex
- W2010943728
- Cited by
- 136
- References
- 116
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 5 · 2023: 5 · 2022: 3 · 2021: 6 · 2020: 6 · 2019: 9 · 2018: 7 · 2017: 8 · 2016: 7 · 2015: 9 · 2014: 8 · 2013: 5 · 2012: 7
- FWCI
- 0.54
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Annals of the New York Academy of Sciences (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Physiology › Nitric Oxide and Endothelin Effects
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Hemoglobin structure and function
- Keywords
- sickle cell disease, reactive oxygen species, free radicals, erythrocytes, oxidative stress, hemoglobin, nitric oxide, vascular function
- MeSH terms
- Anemia, Sickle Cell, Humans, Reactive Oxygen Species
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