Clinical Spectrum of SCID: The Key is in the Thymus? - Wikidata - wikimedia - TriplyDB

Clinical Spectrum of SCID: The Key is in the Thymus?

Clinical Spectrum of SCID: The Key is in the Thymus?

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Unraveling the repertoire in wiskott-Aldrich syndrome

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Clinical Spectrum of SCID: The Key is in the Thymus?

http://www.wikidata.org/entity/Q38343375

description

2014 nî lūn-bûn

@nan

2014年の論文

@ja

2014年論文

@yue

2014年論文

@zh-hant

2014年論文

@zh-hk

2014年論文

@zh-mo

2014年論文

@zh-tw

2014年论文

@wuu

2014年论文

@zh

2014年论文

@zh-cn

name

Clinical Spectrum of SCID: The Key is in the Thymus?

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type

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Clinical Spectrum of SCID: The Key is in the Thymus?

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Clinical Spectrum of SCID: The Key is in the Thymus?

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FIMMU.2014.00111

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Frontiers in Immunology

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Clinical Spectrum of SCID: The Key is in the Thymus?

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Mirjam van der Burg

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P2860

Partial V(D)J recombination activity leads to Omenn syndrome

Homozygous DNA ligase IV R278H mutation in mice leads to leaky SCID and represents a model for human LIG4 syndrome

AIRE deficiency in thymus of 2 patients with Omenn syndrome.

Expansion of immunoglobulin-secreting cells and defects in B cell tolerance in Rag-dependent immunodeficiency.

Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency.

Early defects in human T-cell development severely affect distribution and maturation of thymic stromal cells: possible implications for the pathophysiology of Omenn syndrome

Lymphocyte proliferation in immune-mediated diseases.

Human syndromes of immunodeficiency and dysregulation are characterized by distinct defects in T-cell receptor repertoire development.

More than just SCID--the phenotypic range of combined immunodeficiencies associated with mutations in the recombinase activating genes (RAG) 1 and 2.

A systematic analysis of recombination activity and genotype-phenotype correlation in human recombination-activating gene 1 deficiency.

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111

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scholarly article

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10.3389/FIMMU.2014.00111

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5

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Menno C van Zelm

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2014-03-19T00:00:00Z

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261189384

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24678313

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3958776

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