(Q37394923)

20 August 2017

Graded defects in cytotoxicity determine severity of hemophagocytic lymphohistiocytosis in humans and mice (English)

1 reference

hemophagocytic lymphohistiocytosis

20 August 2017

0 references

1 reference

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

Geneviève de Saint Basile

12 June 2020

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Stephan Ehl

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

author name string

Birthe Jessen

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Tamara Kögl

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Peter Aichele

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

publication date

16 December 2013

1 reference

20 August 2017

Frontiers in Immunology

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

volume

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

page(s)

448

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemo-immunotherapeutic regimens

12 June 2020

cites work

1 reference

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Perforin deficiency impairs a critical immunoregulatory loop involving murine CD8(+) T cells and dendritic cells

6 September 2017

1 reference

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The risk of hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type 2

6 September 2017

1 reference

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Hemophagocytic lymphohistiocytosis in syntaxin-11-deficient mice: T-cell exhaustion limits fatal disease

6 September 2017

1 reference

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Syntaxin 11 is required for NK and CD8⁺ T-cell cytotoxicity and neutrophil degranulation

6 September 2017

1 reference

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Syntaxin-11, but not syntaxin-2 or syntaxin-4, is required for platelet secretion.

6 September 2017

1 reference

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Plasmacytoid dendritic cells are productively infected and activated through TLR-7 early after arenavirus infection

6 September 2017

1 reference

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Platelets support a protective immune response to LCMV by preventing splenic necrosis

6 September 2017

1 reference

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Yet another role for natural killer cells: cytotoxicity in immune regulation and viral persistence

6 September 2017

1 reference

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Natural killer cell activation enhances immune pathology and promotes chronic infection by limiting CD8+ T-cell immunity

6 September 2017

1 reference

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Natural killer cells act as rheostats modulating antiviral T cells

6 September 2017

1 reference

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Critical role for perforin and Fas-dependent killing of dendritic cells in the control of inflammation

6 September 2017

1 reference

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Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH.

6 September 2017

1 reference

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How I treat hemophagocytic lymphohistiocytosis

6 September 2017

1 reference

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Perforin is a critical physiologic regulator of T-cell activation

6 September 2017

1 reference

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X-linked lymphoproliferative disease due to SAP/SH2D1A deficiency: a multicenter study on the manifestations, management and outcome of the disease

6 September 2017

1 reference

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Molecular mechanisms of biogenesis and exocytosis of cytotoxic granules

6 September 2017

1 reference

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Inherited defects in lymphocyte cytotoxic activity

6 September 2017

1 reference

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Neutralization of IFNgamma defeats haemophagocytosis in LCMV-infected perforin- and Rab27a-deficient mice

6 September 2017

1 reference

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Munc18-2 deficiency causes familial hemophagocytic lymphohistiocytosis type 5 and impairs cytotoxic granule exocytosis in patient NK cells

6 September 2017

1 reference

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Familial hemophagocytic lymphohistiocytosis type 5 (FHL-5) is caused by mutations in Munc18-2 and impaired binding to syntaxin 11

6 September 2017

1 reference

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A Griscelli syndrome type 2 murine model of hemophagocytic lymphohistiocytosis (HLH).

6 September 2017

1 reference

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Platelets prevent IFN-alpha/beta-induced lethal hemorrhage promoting CTL-dependent clearance of lymphocytic choriomeningitis virus.

6 September 2017

1 reference

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Novel insights from adaptor protein 3 complex deficiency

6 September 2017

1 reference

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6 September 2017

Hemophagocytic syndromes

1 reference

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Defective cytotoxic lymphocyte degranulation in syntaxin-11 deficient familial hemophagocytic lymphohistiocytosis 4 (FHL4) patients

6 September 2017

1 reference

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Jinx, an MCMV susceptibility phenotype caused by disruption of Unc13d: a mouse model of type 3 familial hemophagocytic lymphohistiocytosis

6 September 2017

1 reference

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Cellular responses to viral infection in humans: lessons from Epstein-Barr virus.

6 September 2017

1 reference

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Intercellular transfer of cell-surface proteins is common and can affect many stages of an immune response

6 September 2017

1 reference

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Familial and acquired hemophagocytic lymphohistiocytosis

6 September 2017

1 reference

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Perforin-mediated target-cell death and immune homeostasis

6 September 2017

1 reference

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Hemophagocytic lymphohistiocytosis and related disorders

6 September 2017

1 reference

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HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

6 September 2017

1 reference

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Genetic disorders of programmed cell death in the immune system

6 September 2017

1 reference

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Perforin-dependent elimination of dendritic cells regulates the expansion of antigen-specific CD8+ T cells in vivo

6 September 2017

1 reference

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NK-DC interaction: on the usefulness of auto-aggression

6 September 2017

1 reference

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Linkage of familial hemophagocytic lymphohistiocytosis (FHL) type-4 to chromosome 6q24 and identification of mutations in syntaxin 11

6 September 2017

1 reference

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Primary hemophagocytic syndromes point to a direct link between lymphocyte cytotoxicity and homeostasis

6 September 2017

1 reference

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Linking albinism and immunity: the secrets of secretory lysosomes

6 September 2017

1 reference

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Evidence of a role for Munc18-2 and microtubules in mast cell granule exocytosis

6 September 2017

1 reference

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Regulation of antigen-specific CD8+ T cell homeostasis by perforin and interferon-gamma.

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Mutations in RAB27A cause Griscelli syndrome associated with haemophagocytic syndrome

6 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Protein truncation test of LYST reveals heterogenous mutations in patients with Chediak-Higashi syndrome

6 September 2017

1 reference

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Perforin gene defects in familial hemophagocytic lymphohistiocytosis.

6 September 2017

1 reference

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Altered trafficking of lysosomal proteins in Hermansky-Pudlak syndrome due to mutations in the beta 3A subunit of the AP-3 adaptor

6 September 2017

1 reference

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Identification of the homologous beige and Chediak-Higashi syndrome genes

6 September 2017

1 reference

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Cytotoxicity mediated by T cells and natural killer cells is greatly impaired in perforin-deficient mice

6 September 2017

1 reference

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Antiviral immune responses of lymphocytic choriomeningitis virus-infected mice lacking CD8+ T lymphocytes because of disruption of the beta 2-microglobulin gene

6 September 2017

1 reference

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Increased susceptibility to cytomegalovirus infection in beige mutant mice

6 September 2017

1 reference

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Susceptibility to lymphocytic choriomeningitis virus isolates correlates directly with early and high cytotoxic T cell activity, as well as with footpad swelling reaction, and all three are regulated by H-2D

6 September 2017

1 reference

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Virus-triggered acquired immunodeficiency by cytotoxic T-cell-dependent destruction of antigen-presenting cells and lymph follicle structure

6 September 2017

1 reference

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Immune response against lymphocytic choriomeningitis virus infection in mice without CD8 expression

6 September 2017

1 reference

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Spectrum of clinical presentations in familial hemophagocytic lymphohistiocytosis type 5 patients with mutations in STXBP2.

6 September 2017

1 reference

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Characteristics of hemophagocytic lymphohistiocytosis in neonates: a nationwide survey in Japan

14 September 2017

1 reference

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An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder

14 September 2017

1 reference

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Feedback regulation of pathogen-specific T cell priming.

14 September 2017

1 reference

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Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation

14 September 2017

1 reference

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Oversecretion of IL-18 in haemophagocytic lymphohistiocytosis: a novel marker of disease activity

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Failure of trafficking and antigen presentation by CD1 in AP-3-deficient cells.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Abnormal expression and subcellular distribution of subunit proteins of the AP-3 adaptor complex lead to platelet storage pool deficiency in the pearl mouse

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Hypercytokinemia in familial hemophagocytic lymphohistiocytosis

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Protection from inflammatory organ damage in a murine model of hemophagocytic lymphohistiocytosis using treatment with IL-18 binding protein

28 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Bifurcation of Toll-Like Receptor 9 Signaling by Adaptor Protein 3

26 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Antigen localisation regulates immune responses in a dose- and time-dependent fashion: a geographical view of immune reactivity

26 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Identification and mutation analysis of the complete gene for Chediak-Higashi syndrome

26 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Cognate peptide-induced destruction of CD8+ cytotoxic T lymphocytes is due to fratricide.

26 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Quantification of lymphocytic choriomeningitis virus with an immunological focus assay in 24- or 96-well plates

26 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Subtle differences in CTL cytotoxicity determine susceptibility to hemophagocytic lymphohistiocytosis in mice and humans with Chediak-Higashi syndrome

26 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH); update 2010.

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Infection of T lymphocytes in Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children of non-Asian origin

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Rab27a regulates phagosomal pH and NADPH oxidase recruitment to dendritic cell phagosomes.

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Spectrum and clinical implications of syntaxin 11 gene mutations in familial haemophagocytic lymphohistiocytosis: association with disease-free remissions and haematopoietic malignancies

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Munc13-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3).

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Mutations associated with neutropenia in dogs and humans disrupt intracellular transport of neutrophil elastase

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

Cutting edge: rapid in vivo killing by memory CD8 T cells

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

A functional and kinetic comparison of antiviral effector and memory cytotoxic T lymphocyte populations in vivo and in vitro.

2 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3864253

A novel single point mutation of the LYST gene in two siblings with different phenotypic features of Chediak Higashi syndrome

2 September 2018

1 reference

12 December 2020

Familial and acquired hemophagocytic lymphohistiocytosis

1 reference

12 December 2020

Molecular analysis and clinical aspects of four patients with Chédiak-Higashi syndrome (CHS)

1 reference

12 December 2020

Platelet secretion defect in patients with familial hemophagocytic lymphohistiocytosis type 5 (FHL-5)

1 reference

12 December 2020

Functional consequences of perforin gene mutations in 22 patients with familial haemophagocytic lymphohistiocytosis

1 reference

12 December 2020

Distinct severity of HLH in both human and murine mutants with complete loss of cytotoxic effector PRF1, RAB27A, and STX11

1 reference

12 December 2020

Familial hemophagocytic lymphohistiocytosis and viral infections

1 reference

12 December 2020

Perforin and Granzymes Have Distinct Roles in Defensive Immunity and Immunopathology

1 reference

12 December 2020

Apparent genotype-phenotype correlation in childhood, adolescent, and adult Chediak-Higashi syndrome

1 reference

12 December 2020

Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis

1 reference

12 December 2020

Lethal hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type II

1 reference

12 December 2020

Identifiers

DOI

10.3389/FIMMU.2013.00448

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

PubMed publication ID

24379813

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:24379813%20AND%20SRC:MED&resulttype=core&format=json