Peripheral expression of hepcidin gene in Egyptian β-thalassemia major

Enein, Azza Aboul; El‐Beshlawy, Amal; Hamdy, Mahmoud; Shaheen, Iman A.; El-Saadany, Zainab Ali; Samir, Ahmed; El-Samie, Hala Abd

doi:10.1016/j.gene.2015.03.048

Cited by 12 publications

(10 citation statements)

References 27 publications

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“…Recently, NTBI and hepcidin have been proposed to be novel reliable markers for iron metabolism, especially, iron overload condition [2,22,23]. Urinary and serum hepcidin levels are decreased in β-thalassemia, which exacerbates the condition leading to further iron overload [24]. Expression of hepcidin in patients with iron overload such as β-thalassemia and myelodysplatic syndromes may be suppressed by the growth differentiation factor 15, the twisted gastrulation factor 1, the bone morphogenetic protein-binding endothelial cell precursor-derived regulator and/or the erythroferrone [2,[25][26][27][28].…”

Section: Introductionmentioning

confidence: 99%

Combined treatment of 3-hydroxypyridine-4-one derivatives and green tea extract to induce hepcidin expression in iron-overloaded β-thalassemic mice

Upanan

Pangjit

Uthaipibull

et al. 2015

Asian Pacific Journal of Tropical Biomedicine

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A B S T R A C T Objective:To evaluate the efficacy of deferiprone (DFP), 1-(N-acetyl-6-aminohexyl)-3-hydroxy-2-methylpyridin-4-one (CM1) or green tea extract (GTE) in enhancing expression of hepatic hepcidin1 (Hamp1) mRNA and relieving iron overload in β-globin knockout thalassemic mice. Methods:The β-globin knockout thalassemic mice were fed with a ferrocene-supplemented diet for 2 months and oral administration of deionized water, DFP (50 mg/kg), CM1 (50 mg/kg), GTE (50 mg epigallocatechin 3-gallate equivalent/kg), GTE along with DFP (50 mg/kg), and GTE along with CM1 (50 mg/kg) every day for 3 months. Levels of hepatic Hamp1 mRNA, plasma non-transferrin bound iron, plasma alanine aminotransferase activity and tissue iron content were determined.Results: All chelation treatments could reduce plasma non-transferrin bound iron concentrations. Additionally, hepatic Hamp1 mRNA expression was significantly up-regulated in the mice in a GTE + DFP combined treatment, correlating with a decrease in the plasma alanine aminotransferase activity and tissue iron deposition. Conclusions:The GTE + DFP treatment could ameliorate iron overload and liver oxidative damage in non-transfusion dependent β-thalassemic mice, by chelating toxic iron in plasma and tissues, and increasing hepcidin expression to inhibit duodenal iron absorption and iron release from hepatocytes and macrophages in the spleen. There is probably an advantage in giving GTE with DFP when treating patients with iron overload.

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Section: Introductionmentioning

confidence: 99%

Combined treatment of 3-hydroxypyridine-4-one derivatives and green tea extract to induce hepcidin expression in iron-overloaded β-thalassemic mice

Upanan

Pangjit

Uthaipibull

et al. 2015

Asian Pacific Journal of Tropical Biomedicine

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“…Hepcidin is a key regulatory factor of iron homeostasis ( 10 , 11 ) in which iron absorption and recycling is affected by the degradation of ferroprotein, thereby downregulating iron concentration in intestinal mucosa cells and macrophage cells and hepatocytes ( 12 ). Currently, iron overload has been identified in several types of chronic diseases due to imbalances in iron homeostasis.…”

Section: Discussionmentioning

confidence: 99%

Hepcidin in non-alcoholic fatty liver disease regulated by the TLR4/NF-κB signaling pathway

Chen

Wang

Huang

et al. 2015

Experimental and Therapeutic Medicine

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The aim of our study was to analyze the role of toll-like receptor 4 (TLR4)/nuclear factor (NF)-κB signal pathway on Hepcidin regulation in non-alcoholic fatty liver disease (NAFLD). A total of 60 male Sprague-Dawley rats were randomly divided into the control, NAFLD and intervention groups. Rats in the control group were fed with standard laboratory diet, and rats in the NAFLD and intervention groups were fed with a high-fat diet. A final volume of 2 ml of pathenolide (10 µmol/l) was administered intraperitoneally only to the rats in the intervention group. The tissue sections were stained with hematoxylin and eosin and the pathological changes in liver tissues were observed and scored. The levels of TLR4 and NF-κB in liver tissues were quantified by western blotting. NAFLD rats appeared to have typical liver fatty degeneration and the expression of TLR4/NF-κB proteins and Hepcidin mRNA was significantly higher than that in the control group (P<0.05). However, the pathological changes observed in the intervention group had a marked improvement with a significant reduction in the TLR4/NF-κB protein and Hepcidin mRNA expression (P<0.05). In conclusion, the abnormal activation of the TLR4/NF-κB signaling pathway may cause NAFLD through the overexpression of Hepcidin.

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“…β-Thalassemia is the most common inherited anemic disorder in Iran; this disorder is caused by mutations in β globin gene. β-Thalassemia-associated anemia occurs due to decreased β globin chain synthesis, increased degradation of available red blood cells (RBC) and short RBC survival ( 1 - 2 ). This disorder is associated with various secondary complications such as extramedullary hematopoiesis, splenomegaly, iron overload, and blood-borne infections.…”

Section: Introductionmentioning

confidence: 99%

“…This disorder is associated with various secondary complications such as extramedullary hematopoiesis, splenomegaly, iron overload, and blood-borne infections. The main cause of mortality in patients with transfusion-dependent thalassemia is iron overload-associated cardiomyopathy 2 . Ferritin is the main form of iron reserve in the body which is found mainly in reticuloendothelial system, hepatocytes, spleen, and bone marrow.…”

Section: Introductionmentioning

confidence: 99%

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Hepcidin and HFE Polymorphisms and Ferritin Level in β-Thalassemia Major

Fekri

Rasouli

Zavareh

et al. 2019

IJHOSCR

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Background: Thalassemia patients need repeated transfusion that lead to increased blood ferritin level and iron overload in the heart and liver. Because the roles of hepcidin antimicrobial peptide (HAMP) and hemocromatosis protein (HFE) in iron metabolism have been confirmed, this study investigated the effects of these gene's polymorphisms on blood ferritin levels and iron overload in the heart and liver in patients with beta thalassemia major Materials and Methods: This cross-sectional study was conducted on 91 patients referring to the Hajar Hospital in Shahrekord, Iran in 2015. After the blood samples were collected, the ferritin levels were measured, DNA was extracted from the blood cells, and the types of polymorphisms were determined using PCR-RFLP. Data of MRI T2* in the heart and liver were drawn from the patients' medical files. Data analysis was conducted by t-test, chi-square test, Fisher's exact test, and Pearson correlation coefficient. Results: There was no significant correlation between blood ferritin level and c.-582 A>G polymorphisms of hepcidin gene (p=0.58), and H63D of HFE gene (p=0.818). In addition, there was no significant association between the polymorphisms and heart and liver MRI, but there was a significant association between blood ferritin level and qualitative heart and liver MRI (r=-0.34, p=0.035 and r=-0.001, p=0.609, respectively). Conclusion: In patients with β-thalassemia major, the presence of c.-582A>G HAMP and H63D HFE polymorphisms is not effective on blood ferritin level and iron overload in the heart and liver in the studied region.

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Peripheral expression of hepcidin gene in Egyptian β-thalassemia major

Cited by 12 publications

References 27 publications

Combined treatment of 3-hydroxypyridine-4-one derivatives and green tea extract to induce hepcidin expression in iron-overloaded β-thalassemic mice

Combined treatment of 3-hydroxypyridine-4-one derivatives and green tea extract to induce hepcidin expression in iron-overloaded β-thalassemic mice

Hepcidin in non-alcoholic fatty liver disease regulated by the TLR4/NF-κB signaling pathway

Hepcidin and HFE Polymorphisms and Ferritin Level in β-Thalassemia Major

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