HSP70 sequestration by free α-globin promotes ineffective erythropoiesis in β-thalassaemia

被引:0
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作者
Jean-Benoît Arlet
Jean-Antoine Ribeil
Flavia Guillem
Olivier Negre
Adonis Hazoume
Guillaume Marcion
Yves Beuzard
Michaël Dussiot
Ivan Cruz Moura
Samuel Demarest
Isaure Chauvot de Beauchêne
Zakia Belaid-Choucair
Margaux Sevin
Thiago Trovati Maciel
Christian Auclair
Philippe Leboulch
Stany Chretien
Luba Tchertanov
Véronique Baudin-Creuza
Renaud Seigneuric
Michaela Fontenay
Carmen Garrido
Olivier Hermine
Geneviève Courtois
机构
[1] Laboratoire INSERM,Département de Biothérapie
[2] unité mixte de recherche 1163,undefined
[3] centre national de la recherche scientifique (CNRS) équipe de recherche labellisée 8254,undefined
[4] 24 Boulevard de Montparnasse,undefined
[5] 75015 Paris,undefined
[6] France,undefined
[7] Service de Médecine Interne,undefined
[8] Faculté de médecine Paris Descartes,undefined
[9] Sorbonne Paris-Cité et Assistance publique – Hôpitaux de Paris,undefined
[10] Hôpital Européen Georges Pompidou,undefined
[11] 15 rue Leblanc 75908 Paris,undefined
[12] France,undefined
[13] Paris Descartes-Sorbonne Paris Cité University,undefined
[14] Imagine Institute,undefined
[15] Assistance publique – Hôpitaux de Paris,undefined
[16] Hôpital Necker,undefined
[17] 24 Boulevard de Montparnasse,undefined
[18] 75015 Paris,undefined
[19] France,undefined
[20] Laboratory of Excellence GR-Ex,undefined
[21] 75015 Paris,undefined
[22] France,undefined
[23] Faculté de médecine Paris Descartes,undefined
[24] Sorbonne Paris-Cité et Assistance publique – Hôpitaux de Paris,undefined
[25] Hôpital Necker,undefined
[26] 149 rue de Sèvres 75015 Paris,undefined
[27] France,undefined
[28] Commissariat à l’énergie atomique (CEA),undefined
[29] Institute of Emerging Diseases and Innovative Therapies (iMETI),undefined
[30] 18 Route du Panorama,undefined
[31] 92260 Fontenay-aux-Roses,undefined
[32] France,undefined
[33] INSERM,undefined
[34] unité mixte de recherche 866,undefined
[35] Equipe labellisée Ligue contre le Cancer and Association pour la Recherche contre le Cancer,undefined
[36] and Laboratoire d’Excellence Lipoprotéines et santé (LipSTIC),undefined
[37] 21033 Dijon,undefined
[38] France,undefined
[39] University of Burgundy,undefined
[40] Faculty of Medicine and Pharmacy,undefined
[41] 7 boulevard Jeanne d'Arc,undefined
[42] 21033 Dijon,undefined
[43] France,undefined
[44] INSERM,undefined
[45] unité mixte de recherche 699,undefined
[46] Hôpital Bichat,undefined
[47] 46 rue Henri Huchard,undefined
[48] 75018 Paris,undefined
[49] France,undefined
[50] Faculté de médecine and Université Denis Diderot Paris VII,undefined
来源
Nature | 2014年 / 514卷
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摘要
In human β-thalassaemiaerythroblasts, HSP70 is sequestered in the cytoplasm by the excess of free α-globin chains and can no longer protect the master transcriptional factor of erythropoiesis GATA-1 from caspase-3 cleavage; transduction of a nuclear-targeted HSP70 or a caspase-3 uncleavable GATA-1 mutant restored maturation of erythropoiesis.
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页码:242 / 246
页数:4
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