Activation of the unfolded protein response is associated with impaired granulopoiesis in transgenic mice expressing mutant Elane.
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The diversity of mutations and clinical outcomes for ELANE-associated neutropeniaThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyInherited bone marrow failure syndromes in adolescents and young adults.Pathogenesis of ELANE-mutant severe neutropenia revealed by induced pluripotent stem cellsCharacterisation of Neutropenia-Associated Neutrophil Elastase Mutations in a Murine Differentiation Model In Vitro and In Vivo.Animal models of human granulocyte diseases.ELANE mutations in cyclic and severe congenital neutropenia: genetics and pathophysiologyExpression of the secondary granule proteins major basic protein 1 (MBP-1) and eosinophil peroxidase (EPX) is required for eosinophilopoiesis in miceA congenital neutrophil defect syndrome associated with mutations in VPS45.Neutropenia-associated ELANE mutations disrupting translation initiation produce novel neutrophil elastase isoforms.Hematopoietic stem cell transplantation for severe congenital neutropenia.Advances in the understanding of Barth syndrome.Cellular stress pathways in pediatric bone marrow failure syndromes: many roads lead to neutropenia.A Truncated Granulocyte Colony-stimulating Factor Receptor (G-CSFR) Inhibits Apoptosis Induced by Neutrophil Elastase G185R Mutant: IMPLICATION FOR UNDERSTANDING CSF3R GENE MUTATIONS IN SEVERE CONGENITAL NEUTROPENIA.How I manage children with neutropenia.Severe congenital neutropenias.A Novel ELANE Mutation Associated with Inflammatory Arthritis, Defective NETosis, and Recurrent Parvoviral Infection.Understanding, treating and avoiding hematological disease: better medicine through mathematics?New monogenic disorders identify more pathways to neutropenia: from the clinic to next-generation sequencing.Neutrophil function in children following allogeneic hematopoietic stem cell transplant.Extended Cleavage Specificity of Human Neutrophil Elastase, Human Proteinase 3, and Their Distant Ortholog Clawed Frog PR3-Three Elastases With Similar Primary but Different Extended Specificities and Stability
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P2860
Activation of the unfolded protein response is associated with impaired granulopoiesis in transgenic mice expressing mutant Elane.
description
2011 nî lūn-bûn
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2011 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
2011年の論文
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2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
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name
Activation of the unfolded pro ...... mice expressing mutant Elane.
@ast
Activation of the unfolded pro ...... mice expressing mutant Elane.
@en
Activation of the unfolded pro ...... mice expressing mutant Elane.
@nl
type
label
Activation of the unfolded pro ...... mice expressing mutant Elane.
@ast
Activation of the unfolded pro ...... mice expressing mutant Elane.
@en
Activation of the unfolded pro ...... mice expressing mutant Elane.
@nl
prefLabel
Activation of the unfolded pro ...... mice expressing mutant Elane.
@ast
Activation of the unfolded pro ...... mice expressing mutant Elane.
@en
Activation of the unfolded pro ...... mice expressing mutant Elane.
@nl
P2093
P2860
P1433
P1476
Activation of the unfolded pro ...... mice expressing mutant Elane.
@en
P2093
Daniel C Link
David S Grenda
Jill Woloszynek
Marie Strand
Mark Murakami
Suparna Nanua
P2860
P304
P356
10.1182/BLOOD-2010-10-311704
P407
P577
2011-02-01T00:00:00Z