Oxidative stress in sickle cell disease

Queiroz, Raphael Ferreira; Lima, Emerson Silva

doi:10.5581/1516-8484.20130008

Cited by 28 publications

(26 citation statements)

References 20 publications

(16 reference statements)

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“…Some hemolytic anemias are also caused by production of surplus ROS . In case of SCD, severe oxidative stress was reported which is also evident from our flow cytometric and microscopic ROS analysis. To have a complete idea of oxidized protein population, we have analyzed the effect of ROS on entire proteome by performing Oxyblot TM analysis.…”

Section: Introductionsupporting

confidence: 75%

2D DIGE based proteomics study of erythrocyte cytosol in sickle cell disease: Altered proteostasis and oxidative stress

et al. 2013

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Sickle cell disease (SCD) is a hemolytic disorder caused by a mutation in beta-globin gene and affects millions of people worldwide. Though clinical manifestations of the disease are quite heterogeneous, many of them occur due to erythrocyte sickling at reduced oxygen concentration and vascular occlusion mediated via blood cell adhesion to the vessel wall. We have followed proteomic approach to resolve the differentially regulated proteins of erythrocyte cytosol. The deregulated proteins mainly fall in the group of chaperone proteins such as heat shock protein 70, alpha hemoglobin stabilizing protein, and redox regulators such as aldehyde dehydrogenase and peroxiredoxin-2 proteoforms. Proteasomal subunits are found to be upregulated and phospho-catalase level also got altered. Severe oxidative stress inside erythrocyte is evident from the ROS analysis and Oxyblot(TM) experiments. Peroxiredoxin-2 shows significant dimerization in the SCD patients, a hallmark of oxidative stress inside erythrocytes. One interesting fact is that most of the differentially regulated proteins are also common for hemoglobinopathies such as Eβ thalassemia. These could provide important clues in understanding the pathophysiology of SCD and lead us to better patient management in the future.

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Section: Introductionsupporting

confidence: 75%

2D DIGE based proteomics study of erythrocyte cytosol in sickle cell disease: Altered proteostasis and oxidative stress

et al. 2013

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“…Although repeated ACS would be predicted to ultimately injure the lung, we suggest that this ACS‐mediated injury may manifest as fibrotic changes in the structural components of the lungs from infarct , rather than directly inducing pulmonary artery remodeling. We suspect the consistent insult from turbulent blood flow , increased inflammation , and oxidative stress at steady state provides a richer environment for the restrictive pulmonary artery remodeling. Likewise, both the thrombotic and proliferative plexiform lesion likely have origins arising from a steady state, rather than acute pathology.…”

Section: Discussionmentioning

confidence: 98%

Pulmonary platelet thrombi and vascular pathology in acute chest syndrome in patients with sickle cell disease

et al. 2016

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A growing body of evidence suggests a role for platelets in sickle cell disease (SCD). Despite the pro-inflammatory, occlusive nature of platelets, a role for platelets in acute chest syndrome (ACS), however, remains understudied. To provide evidence and potentially describe contributory factors for a putative link between ACS and platelets, we performed an autopsy study of 20 SCD cases – 10 of whom died from ACS and 10 whose deaths were not ACS-related. Pulmonary histopathology and case history were collected. We discovered that disseminated pulmonary platelet thrombi were present in 3 out of 10 of cases with ACS, but none of the matched cases without ACS. Those cases with detected thrombi were associated with significant deposition of endothelial vWF and detection of large vWF aggregates adhered to endothelium. Potential clinical risk factors were younger age and higher platelet count at presentation. However, we also noted a sharp and significant decline in platelet count prior to death in each case with platelet thrombi in the lungs. In this study, neither hydroxyurea use nor perimortem transfusion was associated with platelet thrombi. Surprisingly, in all cases, there was profound pulmonary artery remodeling with both thrombotic and proliferative pulmonary plexiform lesions. The severity of remodeling was not associated with a severe history of ACS, or hydroxyurea use, but was inversely correlated with age. We thus provide evidence of undocumented presence of platelet thrombi in cases of fatal ACS describe clinical correlates. We also provide novel correlates of pulmonary remodeling in SCD.

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“…The content of the important antioxidant vitamin E in sickle cell trait erythrocytes are considerably lower than normal and sickle cell trait erythrocytes have increased susceptibility to oxidation [ 21 ]. Recent studies suggest that several mechanisms contribute to the high oxidative burden in sickle cell patients [ 22 , 23 ]. Hypothetically, some of the protective effects of both famine and sickle cell trait in malaria might be attributed to increased premature haemolysis of infected erythrocytes due to oxidative stress.…”

Section: Discussionmentioning

confidence: 99%

Malaria and Malnutrition: Kwashiorkor Associated with Low Levels of Parasitaemia

Fevang

Havemann

Fevang

et al. 2018

Malaria Research and Treatment

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Background The relationship between protein energy malnutrition (PEM) and malaria is controversial. While most studies demonstrate that PEM is associated with greater malaria morbidity, some indicate that PEM may in fact have a protective effect. PEM is differentiated into three subgroups: kwashiorkor (marked protein deficiency), marasmus (calorie deficiency), and kwashiorkor/marasmus. None of the studies concerning PEM and malaria seem to distinguish between these subgroups, and significant differences in susceptibility to malaria between these subgroups may have been overlooked. Plasmodium parasites and malaria infected erythrocytes are sensitive to oxidative stress. Since kwashiorkor patients seem to display an excess of prooxidants and as serum albumin is an important antioxidant, we hypothesized that patients with different forms of PEM might have different levels of malaria parasitaemia. Methods 72 PEM children older than 6 months admitted to Kwale Family Life Training Programme (Kenya) were included in the study. Results Mean parasitaemia was significantly lower in the kwashiorkor group than in the marasmus group (p < 0,001). There was no correlation between serum albumin and parasitaemia. Conclusion Our study suggests a protective effect of kwashiorkor against malaria, warranting further studies.

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Oxidative stress in sickle cell disease

Cited by 28 publications

References 20 publications

2D DIGE based proteomics study of erythrocyte cytosol in sickle cell disease: Altered proteostasis and oxidative stress

2D DIGE based proteomics study of erythrocyte cytosol in sickle cell disease: Altered proteostasis and oxidative stress

Pulmonary platelet thrombi and vascular pathology in acute chest syndrome in patients with sickle cell disease

Malaria and Malnutrition: Kwashiorkor Associated with Low Levels of Parasitaemia

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