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The Neurobeachin-like 2 Protein Regulates Mast Cell Homeostasis

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The Neurobeachin-like 2 Protein Regulates Mast Cell Homeostasis. / Drube, Sebastian; Grimlowski, Randy; Deppermann, Carsten; Fröbel, Julia; Kraft, Florian; Andreas, Nico; Stegner, David; Dudeck, Jan; Weber, Franziska; Rödiger, Mandy; Göpfert, Christiane; Drube, Julia; Reich, Daniela; Nieswandt, Bernhard; Dudeck, Anne; Kamradt, Thomas.

In: J IMMUNOL, Vol. 199, No. 8, 15.10.2017, p. 2948-2957.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Drube, S, Grimlowski, R, Deppermann, C, Fröbel, J, Kraft, F, Andreas, N, Stegner, D, Dudeck, J, Weber, F, Rödiger, M, Göpfert, C, Drube, J, Reich, D, Nieswandt, B, Dudeck, A & Kamradt, T 2017, 'The Neurobeachin-like 2 Protein Regulates Mast Cell Homeostasis', J IMMUNOL, vol. 199, no. 8, pp. 2948-2957. https://doi.org/10.4049/jimmunol.1700556

APA

Drube, S., Grimlowski, R., Deppermann, C., Fröbel, J., Kraft, F., Andreas, N., Stegner, D., Dudeck, J., Weber, F., Rödiger, M., Göpfert, C., Drube, J., Reich, D., Nieswandt, B., Dudeck, A., & Kamradt, T. (2017). The Neurobeachin-like 2 Protein Regulates Mast Cell Homeostasis. J IMMUNOL, 199(8), 2948-2957. https://doi.org/10.4049/jimmunol.1700556

Vancouver

Drube S, Grimlowski R, Deppermann C, Fröbel J, Kraft F, Andreas N et al. The Neurobeachin-like 2 Protein Regulates Mast Cell Homeostasis. J IMMUNOL. 2017 Oct 15;199(8):2948-2957. https://doi.org/10.4049/jimmunol.1700556

Bibtex

@article{b39ca328c2e04d30aa3754961f350f62,
title = "The Neurobeachin-like 2 Protein Regulates Mast Cell Homeostasis",
abstract = "The neurobeachin-like 2 protein (Nbeal2) belongs to the family of beige and Chediak-Higashi (BEACH) domain proteins. Loss-of-function mutations in the human NBEAL2 gene or Nbeal2 deficiency in mice cause gray platelet syndrome, a bleeding disorder characterized by macrothrombocytopenia, splenomegaly, and paucity of α-granules in megakaryocytes and platelets. We found that in mast cells, Nbeal2 regulates the activation of the Shp1-STAT5 signaling axis and the composition of the c-Kit/STAT signalosome. Furthermore, Nbeal2 mediates granule formation and restricts the expression of the transcription factors, IRF8, GATA2, and MITF as well as of the cell-cycle inhibitor p27, which are essential for mast cell differentiation, proliferation, and cytokine production. These data demonstrate the relevance of Nbeal2 in mast cells above and beyond granule biosynthesis.",
keywords = "Animals, Blood Proteins/genetics, Cell Cycle, Cells, Cultured, Cytoplasmic Granules/metabolism, GATA2 Transcription Factor/genetics, Gray Platelet Syndrome/genetics, Hemorrhage, Interferon Regulatory Factors/genetics, Mast Cells/physiology, Megakaryocytes/physiology, Mice, Mice, Knockout, Mutation/genetics, Protein Tyrosine Phosphatase, Non-Receptor Type 6/metabolism, Proto-Oncogene Proteins c-kit/metabolism, STAT5 Transcription Factor/metabolism, Signal Transduction, Splenomegaly, Thrombocytopenia",
author = "Sebastian Drube and Randy Grimlowski and Carsten Deppermann and Julia Fr{\"o}bel and Florian Kraft and Nico Andreas and David Stegner and Jan Dudeck and Franziska Weber and Mandy R{\"o}diger and Christiane G{\"o}pfert and Julia Drube and Daniela Reich and Bernhard Nieswandt and Anne Dudeck and Thomas Kamradt",
note = "Copyright {\textcopyright} 2017 by The American Association of Immunologists, Inc.",
year = "2017",
month = oct,
day = "15",
doi = "10.4049/jimmunol.1700556",
language = "English",
volume = "199",
pages = "2948--2957",
journal = "J IMMUNOL",
issn = "0022-1767",
publisher = "American Association of Immunologists",
number = "8",

}

RIS

TY - JOUR

T1 - The Neurobeachin-like 2 Protein Regulates Mast Cell Homeostasis

AU - Drube, Sebastian

AU - Grimlowski, Randy

AU - Deppermann, Carsten

AU - Fröbel, Julia

AU - Kraft, Florian

AU - Andreas, Nico

AU - Stegner, David

AU - Dudeck, Jan

AU - Weber, Franziska

AU - Rödiger, Mandy

AU - Göpfert, Christiane

AU - Drube, Julia

AU - Reich, Daniela

AU - Nieswandt, Bernhard

AU - Dudeck, Anne

AU - Kamradt, Thomas

N1 - Copyright © 2017 by The American Association of Immunologists, Inc.

PY - 2017/10/15

Y1 - 2017/10/15

N2 - The neurobeachin-like 2 protein (Nbeal2) belongs to the family of beige and Chediak-Higashi (BEACH) domain proteins. Loss-of-function mutations in the human NBEAL2 gene or Nbeal2 deficiency in mice cause gray platelet syndrome, a bleeding disorder characterized by macrothrombocytopenia, splenomegaly, and paucity of α-granules in megakaryocytes and platelets. We found that in mast cells, Nbeal2 regulates the activation of the Shp1-STAT5 signaling axis and the composition of the c-Kit/STAT signalosome. Furthermore, Nbeal2 mediates granule formation and restricts the expression of the transcription factors, IRF8, GATA2, and MITF as well as of the cell-cycle inhibitor p27, which are essential for mast cell differentiation, proliferation, and cytokine production. These data demonstrate the relevance of Nbeal2 in mast cells above and beyond granule biosynthesis.

AB - The neurobeachin-like 2 protein (Nbeal2) belongs to the family of beige and Chediak-Higashi (BEACH) domain proteins. Loss-of-function mutations in the human NBEAL2 gene or Nbeal2 deficiency in mice cause gray platelet syndrome, a bleeding disorder characterized by macrothrombocytopenia, splenomegaly, and paucity of α-granules in megakaryocytes and platelets. We found that in mast cells, Nbeal2 regulates the activation of the Shp1-STAT5 signaling axis and the composition of the c-Kit/STAT signalosome. Furthermore, Nbeal2 mediates granule formation and restricts the expression of the transcription factors, IRF8, GATA2, and MITF as well as of the cell-cycle inhibitor p27, which are essential for mast cell differentiation, proliferation, and cytokine production. These data demonstrate the relevance of Nbeal2 in mast cells above and beyond granule biosynthesis.

KW - Animals

KW - Blood Proteins/genetics

KW - Cell Cycle

KW - Cells, Cultured

KW - Cytoplasmic Granules/metabolism

KW - GATA2 Transcription Factor/genetics

KW - Gray Platelet Syndrome/genetics

KW - Hemorrhage

KW - Interferon Regulatory Factors/genetics

KW - Mast Cells/physiology

KW - Megakaryocytes/physiology

KW - Mice

KW - Mice, Knockout

KW - Mutation/genetics

KW - Protein Tyrosine Phosphatase, Non-Receptor Type 6/metabolism

KW - Proto-Oncogene Proteins c-kit/metabolism

KW - STAT5 Transcription Factor/metabolism

KW - Signal Transduction

KW - Splenomegaly

KW - Thrombocytopenia

U2 - 10.4049/jimmunol.1700556

DO - 10.4049/jimmunol.1700556

M3 - SCORING: Journal article

C2 - 28887433

VL - 199

SP - 2948

EP - 2957

JO - J IMMUNOL

JF - J IMMUNOL

SN - 0022-1767

IS - 8

ER -