XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease - Wikidata - awgldk - TriplyDB

XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease

XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease

http://www.wikidata.org/entity/Q24610800

about

Could the eIF2α-Independent Translation Be the Achilles Heel of Cancer?Regulation of Cell Death by IAPs and Their Antagonists X-linked lymphoproliferative syndromes: brothers or distant cousins?A Nationwide Study of Severe and Protracted Diarrhoea in Patients with Primary Immunodeficiency Diseases Using flow cytometry to screen patients for X-linked lymphoproliferative disease due to SAP deficiency and XIAP deficiency X-linked immunodeficiency with magnesium defect, Epstein-Barr virus infection, and neoplasia disease: a combined immune deficiency with magnesium defect.Whole-exome sequencing reveals overlap between macrophage activation syndrome in systemic juvenile idiopathic arthritis and familial hemophagocytic lymphohistiocytosis Severe viral infections and primary immunodeficiencies.IRES-mediated translation of cellular messenger RNA operates in eIF2α- independent manner during stress.CD27 deficiency is associated with combined immunodeficiency and persistent symptomatic EBV viremia The diagnostic challenge of very early-onset enterocolitis in an infant with XIAP deficiency New monogenic autoinflammatory diseases--a clinical overview Molecular mechanisms in genetically defined autoinflammatory diseases: disorders of amplified danger signaling.Human iNKT and MAIT cells exhibit a PLZF-dependent proapoptotic propensity that is counterbalanced by XIAP Natural killer cell deficiency.Disease-causing mutations in the XIAP BIR2 domain impair NOD2-dependent immune signalling.Caspase-8: not so silently deadly.Familial hemophagocytic lymphohistiocytosis: when rare diseases shed light on immune system functioning.The role of SAP and SLAM family molecules in the humoral immune response.Reduced-intensity conditioning haematopoietic cell transplantation for haemophagocytic lymphohistiocytosis: an important step forward.Insights into NK cell biology from human genetics and disease associations.Familial hemophagocytic lymphohistiocytosis: a model for understanding the human machinery of cellular cytotoxicity.Primary immunodeficiency associated with defects in CD1 and CD1-restricted T cells.Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment.Interleukin-10 and interleukin-10-receptor defects in inflammatory bowel disease.Ubiquitination in disease pathogenesis and treatment.X-linked inhibitor of apoptosis protein deficiency: more than an X-linked lymphoproliferative syndrome.Molecular pathogenesis of lymphomas of mucosa-associated lymphoid tissue--from (auto)antigen driven selection to the activation of NF-κB signaling.Nucleotide-binding oligomerization domain (NOD) signaling defects and cell death susceptibility cannot be uncoupled in X-linked inhibitor of apoptosis (XIAP)-driven inflammatory disease.Macrophage activation syndrome in the era of biologic therapy.Pediatric inflammatory bowel disease: specificity of very early onset.Advances in the pathogenesis of primary and secondary haemophagocytic lymphohistiocytosis: differences and similarities.How genetic testing can lead to targeted management of XIAP deficiency-related inflammatory bowel disease.Severe Epstein-Barr virus infection in primary immunodeficiency and the normal host.Regulating the balance between necroptosis, apoptosis and inflammation by inhibitors of apoptosis proteins.Human immunity against EBV-lessons from the clinic.Sudden unexpected death due to hemophagocytic lymphohistiocytosis syndrome.X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis.Symptomatic males and female carriers in a large Caucasian kindred with XIAP deficiency.Successful allogeneic hematopoietic stem cell transplantation in a boy with X-linked inhibitor of apoptosis deficiency presenting with hemophagocytic lymphohistiocytosis: A case report

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XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease

http://www.wikidata.org/entity/Q24610800

description

2010 nî lūn-bûn

@nan

2010 թուականի Օգոստոսին հրատարակուած գիտական յօդուած

@hyw

2010 թվականի օգոստոսին հրատարակված գիտական հոդված

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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XIAP deficiency: a unique prim ...... ed lymphoproliferative disease

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Alexandra H Filipovich

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Brad McClimon

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Brenda J Kitchen

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Jack J Bleesing

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Kejian Zhang

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Lisa Madden

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Michael B Jordan

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Rajen Mody

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Rebecca A Marsh

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P2860

The X-linked lymphoproliferative-disease gene product SAP regulates signals induced through the co-receptor SLAM

X-linked IAP is a direct inhibitor of cell-death proteases

XIAP, a cellular member of the inhibitor of apoptosis protein family, links the receptors to TAB1-TAK1 in the BMP signaling pathway

A conserved family of cellular genes related to the baculovirus iap gene and encoding apoptosis inhibitors

Inactivating mutations in an SH2 domain-encoding gene in X-linked lymphoproliferative syndrome

XIAP regulates cytosol-specific innate immunity to Listeria infection

Activation of NF-kappa B by XIAP, the X chromosome-linked inhibitor of apoptosis, in endothelial cells involves TAK1

X-linked inhibitor of apoptosis protein functions as a cofactor in transforming growth factor-beta signaling

XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome

Host response to EBV infection in X-linked lymphoproliferative disease results from mutations in an SH2-domain encoding gene

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1079-82

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scholarly article

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44617607

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20489057

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familial hemophagocytic lymphohistiocytosis

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2938130

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