Enhanced oxidative cross-linking of hemoglobin E with spectrin and loss of erythrocyte membrane asymmetry in hemoglobin Eβ-thalassemia

Datta, Poppy; Basu, Sumanta; Chakravarty, Sudipa; Chakravarty, Amit; Banerjee, Debashis; Chandra, Sharmila; Chakrabarti, Abhijit

doi:10.1016/j.bcmd.2006.06.001

Cited by 37 publications

(41 citation statements)

References 33 publications

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“…The HS patients we have studied, perhaps carry dominant mutations in ankyrin, β-spectrin and band 3, drastically affecting PS asymmetry [24]. We have earlier reported higher spectrin binding affinity of α-globin chain compared to β-globin and enhanced yield of spectrin-globin-hydrogen peroxide cross-linked complexes in thalassemia [27,28]. Earlier work from our laboratory also indicated loss of red cell PS asymmetry in different cases of hematological malignancies [29].…”

Section: Discussionmentioning

confidence: 66%

Eryptosis in hereditary spherocytosis and thalassemia: role of glycoconjugates

Basu

Banerjee

Chandra³

et al. 2009

Glycoconj J

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The present work is aimed to study the mechanism of faster erythrocyte clearance in hereditary spherocytosis (HS), a heterogeneous disorders characterized by alterations in the proteins of the red cell membrane skeleton along with different kinds of thalassemia. The maximum exposure of phosphatidylserine (PS) is found in HS compared to those in both α- and β-thalassemia. Interestingly, in HS more PS exposed cells were found in younger erythrocytes compared to normal and the thalassemics where aged cells showed higher loss of PS asymmetry. Loss of sialic acid and GlcNAc bearing glycoconjugates, presumably the glycophorins, was also found upon aging. The loss of PS asymmetry together with the cell surface glycoproteins mediated by membrane vesiculation, seemed to play key role in early clearance of erythrocytes from circulation following a mechanism similar to HbEβ-thalassemia.

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Section: Discussionmentioning

confidence: 66%

Eryptosis in hereditary spherocytosis and thalassemia: role of glycoconjugates

Basu

Banerjee

Chandra³

et al. 2009

Glycoconj J

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“…Além daquelas mencionadas acima, há o aumento de fosfolipídeos e de colesterol, do fluxo de cátions e da permeabilidade ao cálcio. As membranas são mais rígidas e mais instáveis, provavelmente em razão da ligação das cadeias α oxidadas à proteína 4.1 6,93 .…”

Section: Fisiopatologiaunclassified

The genetics of blood disorders: the hereditary hemoglobinopathies

Sonati¹,

Costa²

2008

J Pediatr (Rio J)

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ResumoObjetivo: Sumarizar os dados disponíveis na literatura recente sobre os aspectos fisiopatológicos, de diagnóstico e tratamento das doenças falciformes e da talassemia β, hemoglobinopatias hereditárias de maior relevância nas populações. Fontes dos dados AbstractObjective: To summarize recently published data on the pathophysiology, diagnosis and treatment of sickle cell diseases and β-Thalassemias, the most relevant hereditary hemoglobinopathies in the global population.Sources: Searches were run on the MEDLINE and SCIELO databases, limited to the period from 2003 to May 2008, using the terms hereditary hemoglobinopathies, sickle cell diseases and β-thalassemia. Two books and two chapters were also included. Summary of the findings:More than 2,000 articles were identified; those providing the most important information and broadest views were selected.Conclusions: Morbidity and mortality rates from sickle cell diseases and β-thalassemia are still very high and represent an important challenge. Increased understanding of pathophysiological aspects has lead to significant improvements in treatment and prevention of these diseases.

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“…Importantly, oxidative stress and reactive oxygen species (ROS) are reported to be increased in HbE/b-thal and in b-thal RBC due to excess free a-chains (35,45,160). How HbE exacerbates this oxidative stress is unknown.…”

Section: Fig 2 Hbe Acts As a Bmentioning

confidence: 99%

HbE/β-Thalassemia and Oxidative Stress: The Key to Pathophysiological Mechanisms and Novel Therapeutics

Hirsch

Sibmooh

Fucharoen

et al. 2017

Antioxidants & Redox Signaling

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Significance: Oxidative stress and generation of free radicals are fundamental in initiating pathophysiological mechanisms leading to an inflammatory cascade resulting in high rates of morbidity and death from many inherited point mutation-derived hemoglobinopathies. Hemoglobin (Hb)E is the most common point mutation worldwide. The b E -globin gene is found in greatest frequency in Southeast Asia, including Thailand, Malaysia, Indonesia, Vietnam, Cambodia, and Laos. With the wave of worldwide migration, it is entering the gene pool of diverse populations with greater consequences than expected. Critical Issues: While HbE by itself presents as a mild anemia and a single gene for b-thalassemia is not serious, it remains unexplained why HbE/b-thalassemia (HbE/b-thal) is a grave disease with high morbidity and mortality. Patients often exhibit defective physical development, severe chronic anemia, and often die of cardiovascular disease and severe infections. Recent Advances: This article presents an overview of HbE/b-thal disease with an emphasis on new findings pointing to pathophysiological mechanisms derived from and initiated by the dysfunctional property of HbE as a reduced nitrite reductase concomitant with excess a-chains exacerbating unstable HbE, leading to a combination of nitric oxide imbalance, oxidative stress, and proinflammatory events. Future Directions: Additionally, we present new therapeutic strategies that are based on the emerging molecular-level understanding of the pathophysiology of this and other hemoglobinopathies. These strategies are designed to short-circuit the inflammatory cascade leading to devastating chronic morbidity and fatal consequences. Antioxid. Redox Signal. 26,[794][795][796][797][798][799][800][801][802][803][804][805][806][807][808][809][810][811][812][813]

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Enhanced oxidative cross-linking of hemoglobin E with spectrin and loss of erythrocyte membrane asymmetry in hemoglobin Eβ-thalassemia

Cited by 37 publications

References 33 publications

Eryptosis in hereditary spherocytosis and thalassemia: role of glycoconjugates

Eryptosis in hereditary spherocytosis and thalassemia: role of glycoconjugates

The genetics of blood disorders: the hereditary hemoglobinopathies

HbE/β-Thalassemia and Oxidative Stress: The Key to Pathophysiological Mechanisms and Novel Therapeutics

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