Cited by 136Hemoglobinopathies and Related DisordersNitric Oxide and Endothelin EffectsHemoglobin structure and function

Reactive Species in Sickle Cell Disease

ASLAN, MUTAY, THORNLEY‐BROWN, DENYSE, FREEMAN, BRUCE A.·2000·Annals of the New York Academy of Sciences

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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Crossref · 10.1111/j.1749-6632.2000.tb06201.x

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117 of 117 references verified0 citation errors · 0 not checkable

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Authors
ASLAN, M., THORNLEY‐BROWN, D., & FREEMAN, B. A.
Affiliations
University of Alabama at Birmingham (US)
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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