CEBPE-Mutant Specific Granule Deficiency Correlates With Aberrant Granule Organization and Substantial Proteome Alterations in Neutrophils

CEBPE-Mutant Specific Granule Deficiency Correlates With Aberrant Granule Organization and Substantial Proteome Alterations in Neutrophils

Specific granule deficiency (SGD) is a rare disorder characterized by abnormal neutrophils evidenced by reduced granules, absence of granule proteins, and atypical bilobed nuclei. Mutations in CCAAT/enhancer-binding protein-ε (CEBPE) are one molecular etiology of the disease. Although C/EBPε has bee...

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Bibliographic Details
Main Authors: Nina K. Serwas, Jakob Huemer, Régis Dieckmann, Ester Mejstrikova, Wojciech Garncarz, Jiri Litzman, Birgit Hoeger, Ondrej Zapletal, Ales Janda, Keiryn L. Bennett, Renate Kain, Dontscho Kerjaschky, Kaan Boztug
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-03-01
Series:Frontiers in Immunology
Subjects:
primary immunodeficiency
neutrophil granulocytes
granule organization
C/EBPε
specific granule deficiency
Online Access:http://journal.frontiersin.org/article/10.3389/fimmu.2018.00588/full

Internet

http://journal.frontiersin.org/article/10.3389/fimmu.2018.00588/full

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