-
1
-
-
20444464807
-
Heme-regulated eIF2alpha kinase modifies the phenotypic severity of murine models of erythropoietic protoporphyria and beta-thalassemia
-
Han AP, Fleming MD, Chen JJ. Heme-regulated eIF2alpha kinase modifies the phenotypic severity of murine models of erythropoietic protoporphyria and beta-thalassemia. J Clin Invest 2005; 115:1562-1570.
-
(2005)
J Clin Invest
, vol.115
, pp. 1562-1570
-
-
Han, A.P.1
Fleming, M.D.2
Chen, J.J.3
-
2
-
-
33947584856
-
-
Chen JJ. Regulation of protein synthesis by the heme-regulated eIF2alpha kinase: relevance to anemias. Blood 2007; 109:2693-2699. Heme-regulated translation mediated by the HRI provides one major mechanism that ensures balanced synthesis of globins and heme. Translational regulation by HRI is critical to reduce excess synthesis of globin proteins or heme in thalassemia and other hemoglobinopathies and thus reduces the severity of these diseases.
-
Chen JJ. Regulation of protein synthesis by the heme-regulated eIF2alpha kinase: relevance to anemias. Blood 2007; 109:2693-2699. Heme-regulated translation mediated by the HRI provides one major mechanism that ensures balanced synthesis of globins and heme. Translational regulation by HRI is critical to reduce excess synthesis of globin proteins or heme in thalassemia and other hemoglobinopathies and thus reduces the severity of these diseases.
-
-
-
-
3
-
-
34547699517
-
Foxo3 is required for the regulation of oxidative stress in erythropoiesis
-
The FoxO3 member of the FoxO family of transcription factors is essential to prevent overaccumulation of ROS. In the absence of FoxO3, erythrocytes exhibited decreased expression of ROS-scavenging enzymes and had a ROS-mediated shortened lifespan and evidence of oxidative damage
-
Marinkovic D, Zhang X, Yalcin S, et al. Foxo3 is required for the regulation of oxidative stress in erythropoiesis. J Clin Invest 2007; 117:2133-2144. The FoxO3 member of the FoxO family of transcription factors is essential to prevent overaccumulation of ROS. In the absence of FoxO3, erythrocytes exhibited decreased expression of ROS-scavenging enzymes and had a ROS-mediated shortened lifespan and evidence of oxidative damage.
-
(2007)
J Clin Invest
, vol.117
, pp. 2133-2144
-
-
Marinkovic, D.1
Zhang, X.2
Yalcin, S.3
-
4
-
-
50949133924
-
-
Libani IV, Guy EC, Melchiori L, et al. Decreased differentiation of erythroid cells exacerbates ineffective erythropoiesis in beta-thalassemia. Blood 2008; 112:875-885. This study introduced the notion that decreased erythroid cell differentiation contributes to ineffective erythropoiesis, exacerbating anemia. To some extent, this is mediated by Jak2. This observation may have important consequences as the use of Jak2 inhibitors has the potential to offer an alternative to splenectomy and profoundly change the management of this disorder.
-
Libani IV, Guy EC, Melchiori L, et al. Decreased differentiation of erythroid cells exacerbates ineffective erythropoiesis in beta-thalassemia. Blood 2008; 112:875-885. This study introduced the notion that decreased erythroid cell differentiation contributes to ineffective erythropoiesis, exacerbating anemia. To some extent, this is mediated by Jak2. This observation may have important consequences as the use of Jak2 inhibitors has the potential to offer an alternative to splenectomy and profoundly change the management of this disorder.
-
-
-
-
5
-
-
0004252202
-
-
Cambridge, UK: Cambridge University Press;
-
Steinberg MH, Forget BG, Higgs DR, et al. Disorders of hemoglobin: genetics, pathophysiology and clinical management. Cambridge, UK: Cambridge University Press; 2001.
-
(2001)
Disorders of hemoglobin: Genetics, pathophysiology and clinical management
-
-
Steinberg, M.H.1
Forget, B.G.2
Higgs, D.R.3
-
6
-
-
29744468347
-
Changing patterns of thalassemia worldwide
-
Vichinsky EP. Changing patterns of thalassemia worldwide. Ann N Y Acad Sci 2005; 1054:18-24.
-
(2005)
Ann N Y Acad Sci
, vol.1054
, pp. 18-24
-
-
Vichinsky, E.P.1
-
7
-
-
33846045774
-
HbVar database of human hemoglobin variants and thalassemia mutations: 2007 update
-
Giardine B, van Baal S, Kaimakis P, et al. HbVar database of human hemoglobin variants and thalassemia mutations: 2007 update. Hum Mutat 2007; 28:206.
-
(2007)
Hum Mutat
, vol.28
, pp. 206
-
-
Giardine, B.1
van Baal, S.2
Kaimakis, P.3
-
8
-
-
0004484591
-
Plasma and red cell iron turnover in normal subjects and in patients having various hematopoietic disorders
-
Huff RL, Hennessy TG, Austin RE, et al. Plasma and red cell iron turnover in normal subjects and in patients having various hematopoietic disorders. J Clin Invest 1950; 29:1041-1052.
-
(1950)
J Clin Invest
, vol.29
, pp. 1041-1052
-
-
Huff, R.L.1
Hennessy, T.G.2
Austin, R.E.3
-
9
-
-
0042076793
-
Erythrokinetics in Cooley's anemia
-
Finch CA, Sturgeon P. Erythrokinetics in Cooley's anemia. Blood 1957; 12:64-73.
-
(1957)
Blood
, vol.12
, pp. 64-73
-
-
Finch, C.A.1
Sturgeon, P.2
-
13
-
-
0034669951
-
The importance of erythroid expansion in determining the extent of apoptosis in erythroid precur-sors in patients with beta-thalassemia major
-
Centis F, Tabellini L, Lucarelli G, et al. The importance of erythroid expansion in determining the extent of apoptosis in erythroid precur-sors in patients with beta-thalassemia major. Blood 2000; 96:3624-3629.
-
(2000)
Blood
, vol.96
, pp. 3624-3629
-
-
Centis, F.1
Tabellini, L.2
Lucarelli, G.3
-
14
-
-
0036180342
-
Pathophysiology of thalassemia
-
Schrier SL. Pathophysiology of thalassemia. Curr Opin Hematol 2002; 9:123-126.
-
(2002)
Curr Opin Hematol
, vol.9
, pp. 123-126
-
-
Schrier, S.L.1
-
15
-
-
41449094655
-
Stroke in thalassemia: A dilemma
-
This article underlines recent observations that suggest a potential correlation between splenectomy and stroke in beta-thalassemia
-
Taher A, Mehio G, Isma'eel H, et al. Stroke in thalassemia: a dilemma. Am J Hematol 2008; 83:343. This article underlines recent observations that suggest a potential correlation between splenectomy and stroke in beta-thalassemia.
-
(2008)
Am J Hematol
, vol.83
, pp. 343
-
-
Taher, A.1
Mehio, G.2
Isma'eel, H.3
-
16
-
-
0018614007
-
Iron absorption and loading in beta-thalassaemia intermedia
-
Pippard MJ, Callender ST, Warner GT, et al. Iron absorption and loading in beta-thalassaemia intermedia. Lancet 1979; 2:819-821.
-
(1979)
Lancet
, vol.2
, pp. 819-821
-
-
Pippard, M.J.1
Callender, S.T.2
Warner, G.T.3
-
17
-
-
0019944568
-
Ferrioxamine excretion in iron-loaded man
-
Pippard MJ, Callender ST, Finch CA. Ferrioxamine excretion in iron-loaded man. Blood 1982; 60:288-294.
-
(1982)
Blood
, vol.60
, pp. 288-294
-
-
Pippard, M.J.1
Callender, S.T.2
Finch, C.A.3
-
18
-
-
4444274916
-
Labile plasma iron (LPI) as an indicator of chelatable plasma redox activity in iron-overloaded beta-thalassemia/ HbE patients treated with an oral chelator
-
Pootrakul P, Breuer W, Sametband M, et al. Labile plasma iron (LPI) as an indicator of chelatable plasma redox activity in iron-overloaded beta-thalassemia/ HbE patients treated with an oral chelator. Blood 2004; 104:1504-1510.
-
(2004)
Blood
, vol.104
, pp. 1504-1510
-
-
Pootrakul, P.1
Breuer, W.2
Sametband, M.3
-
19
-
-
29744461403
-
Role of iron in inducing oxidative stress in thalassemia: Can it be prevented by inhibition of absorption and by antioxidants?
-
Rachmilewitz EA, Weizer-Stern O, Adamsky K, et al. Role of iron in inducing oxidative stress in thalassemia: can it be prevented by inhibition of absorption and by antioxidants? Ann N Y Acad Sci 2005; 1054:118-123.
-
(2005)
Ann N Y Acad Sci
, vol.1054
, pp. 118-123
-
-
Rachmilewitz, E.A.1
Weizer-Stern, O.2
Adamsky, K.3
-
20
-
-
0017622851
-
Continuous subcutaenous administration of deferoxamine in patients with iron overload
-
Propper RD, Cooper B, Rufo RR, et al. Continuous subcutaenous administration of deferoxamine in patients with iron overload. N Engl J Med 1977; 297:418-423.
-
(1977)
N Engl J Med
, vol.297
, pp. 418-423
-
-
Propper, R.D.1
Cooper, B.2
Rufo, R.R.3
-
21
-
-
12344326653
-
Clinical aspects of beta-thalassemia
-
Steinberg MH, Forget BG, Higgs DR, Nagel RL, editors, Cambridge, England: Cambridge University Press;
-
Olivieri NF, Weatherall DJ. Clinical aspects of beta-thalassemia. In: Steinberg MH, Forget BG, Higgs DR, Nagel RL, editors. Disorders of hemoglobin: genetics, pathophysiology and clinical management. Cambridge, England: Cambridge University Press; 2001.
-
(2001)
Disorders of hemoglobin: Genetics, pathophysiology and clinical management
-
-
Olivieri, N.F.1
Weatherall, D.J.2
-
22
-
-
0014061434
-
Thalassemia intermedia, with iron overload, cardiac failure, diabetes mellitus, hypopituitarism and porphyrinuria
-
Bannerman RM, Keusch G, Kreimer-Birnbaum M, et al. Thalassemia intermedia, with iron overload, cardiac failure, diabetes mellitus, hypopituitarism and porphyrinuria. Am J Med 1967; 42:476-486.
-
(1967)
Am J Med
, vol.42
, pp. 476-486
-
-
Bannerman, R.M.1
Keusch, G.2
Kreimer-Birnbaum, M.3
-
23
-
-
0019792931
-
Iron overload and desferrioxamine chelation therapy in beta-thalassemia intermedia
-
Cossu P, Toccafondi C, Vardeu F, et al. Iron overload and desferrioxamine chelation therapy in beta-thalassemia intermedia. Eur J Pediatr 1981; 137:267-271.
-
(1981)
Eur J Pediatr
, vol.137
, pp. 267-271
-
-
Cossu, P.1
Toccafondi, C.2
Vardeu, F.3
-
25
-
-
0018773439
-
Mechanism of desferrioxamine-induced iron excretion in thalassaemia
-
Hershko C, Rachmilewitz EA. Mechanism of desferrioxamine-induced iron excretion in thalassaemia. Br J Haematol 1979; 42:125-132.
-
(1979)
Br J Haematol
, vol.42
, pp. 125-132
-
-
Hershko, C.1
Rachmilewitz, E.A.2
-
26
-
-
0025363102
-
Iron overload in Chinese patients with hemoglobin H disease
-
Hsu HC, Lin CK, Tsay SH, et al. Iron overload in Chinese patients with hemoglobin H disease. Am J Hematol 1990; 34:287-290.
-
(1990)
Am J Hematol
, vol.34
, pp. 287-290
-
-
Hsu, H.C.1
Lin, C.K.2
Tsay, S.H.3
-
27
-
-
0001369958
-
Studies on congenital hemolytic syndromes. Part IV: Gastrointestinal absorption of iron
-
Erlandson ME, Walden B, Stern G, et al. Studies on congenital hemolytic syndromes. Part IV: gastrointestinal absorption of iron. Blood 1962; 19:359-378.
-
(1962)
Blood
, vol.19
, pp. 359-378
-
-
Erlandson, M.E.1
Walden, B.2
Stern, G.3
-
28
-
-
0023100003
-
Erythroid marrow function in anemic patients
-
Cazzola M, Pootrakul P, Huebers HA, et al. Erythroid marrow function in anemic patients. Blood 1987; 69:296-301.
-
(1987)
Blood
, vol.69
, pp. 296-301
-
-
Cazzola, M.1
Pootrakul, P.2
Huebers, H.A.3
-
29
-
-
0028049495
-
Regulators of iron balance in humans
-
Finch C. Regulators of iron balance in humans. Blood 1994; 84:1697-1702.
-
(1994)
Blood
, vol.84
, pp. 1697-1702
-
-
Finch, C.1
-
30
-
-
0035902605
-
Lack of hepcidin gene expression and severe tissue iron overload in upstream stimulatory factor 2 (USF2) knockout mice
-
Nicolas G, Bennoun M, Devaux I, et al. Lack of hepcidin gene expression and severe tissue iron overload in upstream stimulatory factor 2 (USF2) knockout mice. Proc Natl Acad Sci U S A 2001; 98:8780-8785.
-
(2001)
Proc Natl Acad Sci U S A
, vol.98
, pp. 8780-8785
-
-
Nicolas, G.1
Bennoun, M.2
Devaux, I.3
-
31
-
-
10844258104
-
Hepcidin regulates cellular iron efflux by binding to ferroportin and inducing its internalization
-
Nemeth E, Tuttle MS, Powelson J, et al. Hepcidin regulates cellular iron efflux by binding to ferroportin and inducing its internalization. Science 2004; 306:2090-2093.
-
(2004)
Science
, vol.306
, pp. 2090-2093
-
-
Nemeth, E.1
Tuttle, M.S.2
Powelson, J.3
-
32
-
-
33845245942
-
Suppression of hepcidin during anemia requires erythropoietic activity
-
Pak M, Lopez MA, Gabayan V, et al. Suppression of hepcidin during anemia requires erythropoietic activity. Blood 2006; 108:3730-3735.
-
(2006)
Blood
, vol.108
, pp. 3730-3735
-
-
Pak, M.1
Lopez, M.A.2
Gabayan, V.3
-
33
-
-
34447120059
-
-
Peyssonnaux C, Zinkernagel AS, Schuepbach RA, et al. Regulation of iron homeostasis by the hypoxia-inducible transcription factors (HIFs). J Clin Invest 2007; 117:1926-1932. This work shows that the von Hippel-Lindau/hypoxia- inducible transcription factor (VHL/HIF) pathway is an essential link between iron homeostasis and hepcidin regulation in vivo.
-
Peyssonnaux C, Zinkernagel AS, Schuepbach RA, et al. Regulation of iron homeostasis by the hypoxia-inducible transcription factors (HIFs). J Clin Invest 2007; 117:1926-1932. This work shows that the von Hippel-Lindau/hypoxia- inducible transcription factor (VHL/HIF) pathway is an essential link between iron homeostasis and hepcidin regulation in vivo.
-
-
-
-
34
-
-
47049116150
-
Erythropoietin mediates hepcidin expression in hepatocytes through EPOR signalling and regulation of C/EBP{alpha}
-
This study suggests a direct involvement of Epo in hepcidin regulation through the transcriptional factor C/EBPalpha
-
Pinto JP, Ribeiro S, Pontes H, et al. Erythropoietin mediates hepcidin expression in hepatocytes through EPOR signalling and regulation of C/EBP{alpha}. Blood 2008; 111:5727-5733. This study suggests a direct involvement of Epo in hepcidin regulation through the transcriptional factor C/EBPalpha.
-
(2008)
Blood
, vol.111
, pp. 5727-5733
-
-
Pinto, J.P.1
Ribeiro, S.2
Pontes, H.3
-
35
-
-
34948904750
-
High levels of GDF15 in thalassemia suppress expression of the iron regulatory protein hepcidin
-
This study showed that a protein called GDF15 is secreted by thalassemic red cells suppressing hepcidin expression in the liver. This observation may have important consequences as GDF15 might be utilized as a scientific and therapeutic tool to investigate ineffective erythropoiesis and iron metabolism
-
Tanno T, Bhanu NV, Oneal PA, et al. High levels of GDF15 in thalassemia suppress expression of the iron regulatory protein hepcidin. Nat Med 2007; 13:1096-1101. This study showed that a protein called GDF15 is secreted by thalassemic red cells suppressing hepcidin expression in the liver. This observation may have important consequences as GDF15 might be utilized as a scientific and therapeutic tool to investigate ineffective erythropoiesis and iron metabolism.
-
(2007)
Nat Med
, vol.13
, pp. 1096-1101
-
-
Tanno, T.1
Bhanu, N.V.2
Oneal, P.A.3
-
36
-
-
58149399349
-
Elevated growth differentiation factor 15 expression in patients with congenital dyserythropoietic anemia type I
-
Tamary H, Shalev H, Perez-Avraham G, et al. Elevated growth differentiation factor 15 expression in patients with congenital dyserythropoietic anemia type I. Blood 2008; 112:5241-5244.
-
(2008)
Blood
, vol.112
, pp. 5241-5244
-
-
Tamary, H.1
Shalev, H.2
Perez-Avraham, G.3
-
37
-
-
59249095557
-
Regulation of iron absorption in hemoglobinopathies
-
This is a comprehensive review of mouse models of thalassemia and sickle cell anemia and how they have been utilized to study the relationship between erythropoiesis and iron metabolism
-
Rechavi G, Rivella S. Regulation of iron absorption in hemoglobinopathies. Curr Mol Med 2008; 8:646-662. This is a comprehensive review of mouse models of thalassemia and sickle cell anemia and how they have been utilized to study the relationship between erythropoiesis and iron metabolism.
-
(2008)
Curr Mol Med
, vol.8
, pp. 646-662
-
-
Rechavi, G.1
Rivella, S.2
-
38
-
-
33845342174
-
Urinary hepcidin in congenital chronic anemias
-
This study determined the hepcidin levels in patients with congenital chronic anemias, including thalassemic patients
-
Kearney SL, Nemeth E, Neufeld EJ, et al. Urinary hepcidin in congenital chronic anemias. Pediatr Blood Cancer 2007; 48:57-63. This study determined the hepcidin levels in patients with congenital chronic anemias, including thalassemic patients.
-
(2007)
Pediatr Blood Cancer
, vol.48
, pp. 57-63
-
-
Kearney, S.L.1
Nemeth, E.2
Neufeld, E.J.3
-
39
-
-
34447306076
-
Liver iron concentrations and urinary hepcidin in beta-thalassemia
-
This study determined the hepcidin levels in patients with thalassemic intermedia and major, showing a strong correlation between high erythropoietic drive and severe hepcidin deficiency
-
Origa R, Galanello R, Ganz T, et al. Liver iron concentrations and urinary hepcidin in beta-thalassemia. Haematologica 2007; 92:583-588. This study determined the hepcidin levels in patients with thalassemic intermedia and major, showing a strong correlation between high erythropoietic drive and severe hepcidin deficiency.
-
(2007)
Haematologica
, vol.92
, pp. 583-588
-
-
Origa, R.1
Galanello, R.2
Ganz, T.3
-
40
-
-
0346433908
-
Decreased hepcidin mRNA expression in thalassemic mice
-
Adamsky K, Weizer O, Amariglio N, et al. Decreased hepcidin mRNA expression in thalassemic mice. Br J Haematol 2004; 124:123-124.
-
(2004)
Br J Haematol
, vol.124
, pp. 123-124
-
-
Adamsky, K.1
Weizer, O.2
Amariglio, N.3
-
41
-
-
29744463634
-
Exploring the role of hepcidin, an antimicrobial and iron regulatory peptide, in increased iron absorption in beta-thalassemia
-
Breda L, Gardenghi S, Guy E, et al. Exploring the role of hepcidin, an antimicrobial and iron regulatory peptide, in increased iron absorption in beta-thalassemia. Ann N Y Acad Sci 2005; 1054:417-422.
-
(2005)
Ann N Y Acad Sci
, vol.1054
, pp. 417-422
-
-
Breda, L.1
Gardenghi, S.2
Guy, E.3
-
42
-
-
33750033613
-
Liver expression of hepcidin and other iron genes in two mouse models of beta-thalassemia
-
De Franceschi L, Daraio F, Filippini A, et al. Liver expression of hepcidin and other iron genes in two mouse models of beta-thalassemia. Haematologica 2006; 91:1336-1342.
-
(2006)
Haematologica
, vol.91
, pp. 1336-1342
-
-
De Franceschi, L.1
Daraio, F.2
Filippini, A.3
-
43
-
-
34249658982
-
Ineffective erythropoiesis in {beta}-thalassemia is characterized by increased iron absorption mediated by down-regulation of hepcidin and up-regulation of ferroportin
-
This study reveals that the degree of ineffective erythropoiesis dictates tissue iron distribution and that ineffective erythropoiesis and iron content regulate hepcidin and other iron-regulatory genes such as Hfe and Cebpa
-
Gardenghi S, Marongiu MF, Ramos P, et al. Ineffective erythropoiesis in {beta}-thalassemia is characterized by increased iron absorption mediated by down-regulation of hepcidin and up-regulation of ferroportin. Blood 2007; 109:5027-5035. This study reveals that the degree of ineffective erythropoiesis dictates tissue iron distribution and that ineffective erythropoiesis and iron content regulate hepcidin and other iron-regulatory genes such as Hfe and Cebpa.
-
(2007)
Blood
, vol.109
, pp. 5027-5035
-
-
Gardenghi, S.1
Marongiu, M.F.2
Ramos, P.3
-
44
-
-
0037606048
-
A novel murine model of Cooley anemia and its rescue by lentiviral-mediated human beta -globin gene transfer
-
Rivella S, May C, Chadburn A, et al. A novel murine model of Cooley anemia and its rescue by lentiviral-mediated human beta -globin gene transfer. Blood 2003; 101:2932-2939.
-
(2003)
Blood
, vol.101
, pp. 2932-2939
-
-
Rivella, S.1
May, C.2
Chadburn, A.3
-
45
-
-
34347389655
-
Iron homeostasis during transfusional iron overload in beta-thalassemia and sickle cell disease: Changes in iron regulatory protein, hepcidin, and ferritin expression
-
This study indicates that in patients affected by beta-thalassemia in which the erythropoietic drive was relieved by transfusion, the hepcidin levels are high, purely reflecting the response of this hormone to excess liver iron
-
Jenkins ZA, Hagar W, Bowlus CL, et al. Iron homeostasis during transfusional iron overload in beta-thalassemia and sickle cell disease: changes in iron regulatory protein, hepcidin, and ferritin expression. Pediatr Hematol Oncol 2007; 24:237-243. This study indicates that in patients affected by beta-thalassemia in which the erythropoietic drive was relieved by transfusion, the hepcidin levels are high, purely reflecting the response of this hormone to excess liver iron.
-
(2007)
Pediatr Hematol Oncol
, vol.24
, pp. 237-243
-
-
Jenkins, Z.A.1
Hagar, W.2
Bowlus, C.L.3
-
46
-
-
33745755350
-
The effects of erythropoietic activity and iron burden on hepcidin expression in patients with thalassemia major
-
Kattamis A, Papassotiriou I, Palaiologou D, et al. The effects of erythropoietic activity and iron burden on hepcidin expression in patients with thalassemia major. Haematologica 2006; 91:809-812.
-
(2006)
Haematologica
, vol.91
, pp. 809-812
-
-
Kattamis, A.1
Papassotiriou, I.2
Palaiologou, D.3
-
47
-
-
45349107618
-
Fetal globin stimulant therapies in the beta- hemoglobinopathies: Principles and current potential
-
Perrine SP. Fetal globin stimulant therapies in the beta- hemoglobinopathies: principles and current potential. Pediatr Ann 2008; 37:339-346.
-
(2008)
Pediatr Ann
, vol.37
, pp. 339-346
-
-
Perrine, S.P.1
-
48
-
-
45149113001
-
JAK2 mutations are present in all cases of polycythemia vera
-
Wang YL, Vandris K, Jones A, et al. JAK2 mutations are present in all cases of polycythemia vera. Leukemia 2007; 22:1289.
-
(2007)
Leukemia
, vol.22
, pp. 1289
-
-
Wang, Y.L.1
Vandris, K.2
Jones, A.3
-
49
-
-
59249091317
-
The role of oxidative stress in hemolytic anemia
-
A comprehensive review of the role of ROS in hemolytic anemias
-
Fibach E, Rachmilewitz E. The role of oxidative stress in hemolytic anemia. Curr Mol Med 2008; 8:609-619. A comprehensive review of the role of ROS in hemolytic anemias.
-
(2008)
Curr Mol Med
, vol.8
, pp. 609-619
-
-
Fibach, E.1
Rachmilewitz, E.2
-
50
-
-
0037331669
-
Flow cytometric measurement of reactive oxygen species production by normal and thalassaemic red blood cells
-
Amer J, Goldfarb A, Fibach E. Flow cytometric measurement of reactive oxygen species production by normal and thalassaemic red blood cells. Eur J Haematol 2003; 70:84-90.
-
(2003)
Eur J Haematol
, vol.70
, pp. 84-90
-
-
Amer, J.1
Goldfarb, A.2
Fibach, E.3
-
51
-
-
38549144785
-
-
Amer J, Atlas D, Fibach E. N-acetylcysteine amide (AD4) attenuates oxidative stress in beta-thalassemia blood cells. Biochim Biophys Acta 2008; 1780:249-255. This article describes that patients affected by sickle cell anemia and thalassemia exhibited 10-fold to 30-fold higher ROS production and 20-50% lower GSH content in RBC, platelets, and polymorphonuclear cells (PMN) compared with normal counterparts. These results suggest that these patients may benefit from antioxidant therapies, limiting oxidative damage to RBC, PMN, and platelets and potentially alleviating symptoms associated with their pathology.
-
Amer J, Atlas D, Fibach E. N-acetylcysteine amide (AD4) attenuates oxidative stress in beta-thalassemia blood cells. Biochim Biophys Acta 2008; 1780:249-255. This article describes that patients affected by sickle cell anemia and thalassemia exhibited 10-fold to 30-fold higher ROS production and 20-50% lower GSH content in RBC, platelets, and polymorphonuclear cells (PMN) compared with normal counterparts. These results suggest that these patients may benefit from antioxidant therapies, limiting oxidative damage to RBC, PMN, and platelets and potentially alleviating symptoms associated with their pathology.
-
-
-
-
52
-
-
51349117161
-
Oxidative stress in the regulation of normal and neoplastic hematopoiesis
-
A thorough review of the role of FoxO family members in regulating hematopoietic cell homeostasis
-
Ghaffari S. Oxidative stress in the regulation of normal and neoplastic hematopoiesis. Antioxid Redox Signal 2008; 10:1923-1940. A thorough review of the role of FoxO family members in regulating hematopoietic cell homeostasis.
-
(2008)
Antioxid Redox Signal
, vol.10
, pp. 1923-1940
-
-
Ghaffari, S.1
-
53
-
-
55749096751
-
-
Kerenyi MA, Grebien F, Gehart H, et al. Stat5 regulates cellular iron uptake of erythroid cells via IRP-2 and TfR-1. Blood 2008; 112:3878-3888. This study suggests a link between EpoR/Jak/Stat signaling and iron metabolism, showing that in mice that completely lack Stat5 activity the cell surface levels of TfR-1 on erythroid cells were decreased more than 2-fold. The authors showed that reduction could be attributed to reduced transcription of TfR-1 mRNA and iron regulatory protein 2 (IRP-2), the major translational regulator of TfR-1 mRNA stability in erythroid cells.
-
Kerenyi MA, Grebien F, Gehart H, et al. Stat5 regulates cellular iron uptake of erythroid cells via IRP-2 and TfR-1. Blood 2008; 112:3878-3888. This study suggests a link between EpoR/Jak/Stat signaling and iron metabolism, showing that in mice that completely lack Stat5 activity the cell surface levels of TfR-1 on erythroid cells were decreased more than 2-fold. The authors showed that reduction could be attributed to reduced transcription of TfR-1 mRNA and iron regulatory protein 2 (IRP-2), the major translational regulator of TfR-1 mRNA stability in erythroid cells.
-
-
-
-
54
-
-
62649135982
-
-
De Domenico I, Lo E, Ward DM, Kaplan J. Hepcidin-induced internalization of ferroportin requires binding and cooperative interaction with Jak2. Proc Natl Acad Sci USA 2009; 106:3800-3805. This study links Jak2 to ferroportin. Hepcidin binds to the iron exporter ferroportin, inducing its degradation and thus preventing iron entry into plasma. This paper indicates that Jak2 phosphorylates ferroportin, following binding of this protein to hepcidin. Phosphorylation of ferroportin then triggers its internalization and degradation.
-
De Domenico I, Lo E, Ward DM, Kaplan J. Hepcidin-induced internalization of ferroportin requires binding and cooperative interaction with Jak2. Proc Natl Acad Sci USA 2009; 106:3800-3805. This study links Jak2 to ferroportin. Hepcidin binds to the iron exporter ferroportin, inducing its degradation and thus preventing iron entry into plasma. This paper indicates that Jak2 phosphorylates ferroportin, following binding of this protein to hepcidin. Phosphorylation of ferroportin then triggers its internalization and degradation.
-
-
-
|