Genetic defects in severe congenital neutropenia: emerging insights into life and death of human neutrophil granulocytes
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JAGN1 deficiency causes aberrant myeloid cell homeostasis and congenital neutropeniaNeutrophil biology: an updateFrom cytopenia to leukemia: the role of Gfi1 and Gfi1b in blood formationB cell-helper neutrophils stimulate the diversification and production of immunoglobulin in the marginal zone of the spleenJagunal homolog 1 is a critical regulator of neutrophil function in fungal host defenseHuman RHOH deficiency causes T cell defects and susceptibility to EV-HPV infectionsA novel homozygous mutation in G6PC3 presenting as cyclic neutropenia and severe congenital neutropenia in the same family.Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib.Validation is a dish oft served coldInherited biallelic CSF3R mutations in severe congenital neutropenia.Neutrophil-mediated phagocytic host defense defect in myeloid Cftr-inactivated mice.Evaluation and management of patients with isolated neutropeniaThe antimicrobial propeptide hCAP-18 plasma levels in neutropenia of various aetiologies: a prospective study.Neutrophil depletion impairs natural killer cell maturation, function, and homeostasis.The phenotype of human STK4 deficiency.Non-steady-state hematopoiesis regulated by the C/EBPβ transcription factor.Characterisation of Neutropenia-Associated Neutrophil Elastase Mutations in a Murine Differentiation Model In Vitro and In Vivo.Nuclear-to-cytoplasmic relocalization of the proliferating cell nuclear antigen (PCNA) during differentiation involves a chromosome region maintenance 1 (CRM1)-dependent export and is a prerequisite for PCNA antiapoptotic activity in mature neutrophAdvances in genetic studies of inherited bone marrow failure syndromes and their associated malignancies.ELANE mutations in cyclic and severe congenital neutropenia: genetics and pathophysiologyImmunoblotting validation of research antibodies generated against HS1-associated protein X-1 in the human neutrophil model cell line PLB-985Expression of the secondary granule proteins major basic protein 1 (MBP-1) and eosinophil peroxidase (EPX) is required for eosinophilopoiesis in miceGenetic predispositions to childhood leukemia.A congenital neutrophil defect syndrome associated with mutations in VPS45.The soluble pattern recognition receptor PTX3 links humoral innate and adaptive immune responses by helping marginal zone B cells.Recent insights into inherited bone marrow failure syndromes.Educational paper: Defects in number and function of neutrophilic granulocytes causing primary immunodeficiency.Hematopoietic stem cell transplantation for severe congenital neutropenia.Acute lung injury results from failure of neutrophil de-priming: a new hypothesis.ICON: the early diagnosis of congenital immunodeficiencies.Important differences in the diagnostic spectrum of primary immunodeficiency in adults versus children.Severe combined immunodeficiency--an update.Understanding chronic neutropenia: life is short.How we diagnose and treat neutropenia in adults.Bcl-2 proteins in development, health, and disease of the hematopoietic system.Proliferating cell nuclear antigen in neutrophil fate.Kostmann's Disease and HCLS1-Associated Protein X-1 (HAX1).Survival and differentiation defects contribute to neutropenia in glucose-6-phosphatase-β (G6PC3) deficiency in a model of mouse neutrophil granulocyte differentiation.How I manage children with neutropenia.A unified pathogenesis for kidney diseases, including genetic diseases and cancers, by the protein-homeostasis-system hypothesis
P2860
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P2860
Genetic defects in severe congenital neutropenia: emerging insights into life and death of human neutrophil granulocytes
description
2011 nî lūn-bûn
@nan
2011 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
2011年の論文
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2011年学术文章
@wuu
2011年学术文章
@zh-cn
2011年学术文章
@zh-hans
2011年学术文章
@zh-my
2011年学术文章
@zh-sg
2011年學術文章
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name
Genetic defects in severe cong ...... human neutrophil granulocytes
@ast
Genetic defects in severe cong ...... human neutrophil granulocytes
@en
Genetic defects in severe cong ...... human neutrophil granulocytes
@nl
type
label
Genetic defects in severe cong ...... human neutrophil granulocytes
@ast
Genetic defects in severe cong ...... human neutrophil granulocytes
@en
Genetic defects in severe cong ...... human neutrophil granulocytes
@nl
altLabel
Genetic Defects in Severe Cong ...... Human Neutrophil Granulocytes
@en
prefLabel
Genetic defects in severe cong ...... human neutrophil granulocytes
@ast
Genetic defects in severe cong ...... human neutrophil granulocytes
@en
Genetic defects in severe cong ...... human neutrophil granulocytes
@nl
P1476
Genetic Defects in Severe Cong ...... Human Neutrophil Granulocytes
@en
Genetic defects in severe cong ...... human neutrophil granulocytes
@en
P2093
Christoph Klein
P304
P356
10.1146/ANNUREV-IMMUNOL-030409-101259
P407
P577
2011-01-01T00:00:00Z
2011-04-23T00:00:00Z