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

被引：0

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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中图分类号：

学科分类号：

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.

引用

页码：242 / 246

页数：4

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