Characterisation of Neutropenia-Associated Neutrophil Elastase Mutations in a Murine Differentiation Model In Vitro and In Vivo

Severe congenital neutropenia (SCN) is characterised by a differentiation block in the bone marrow and low neutrophil numbers in the peripheral blood, which correlates with increased risk of bacterial infections. Several underlying gene defects have been identified in SCN patients. Mutations in the...

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Veröffentlicht in:	PloS one 2016-12, Vol.11 (12), p.e0168055-e0168055
Hauptverfasser:	Wiesmeier, Michael, Gautam, Sanjivan, Kirschnek, Susanne, Häcker, Georg
Format:	Artikel
Sprache:	eng
Schlagworte:	Adoptive transfer Analysis Animal models Animals Apoptosis Bacteria Bacterial diseases Bacterial infections Biology and Life Sciences Bone marrow Cell death Cell Survival Congenital diseases Defects Differentiation Disease Models, Animal Elastase Evacuations & rescues Gene mutation Genes Genetic aspects Glucose Granulopoiesis Health aspects Health risks HEK293 Cells Homeodomain Proteins - genetics Humans Hygiene Hypotheses Kinases Leukocyte Elastase - deficiency Leukocyte Elastase - genetics Leukocytes (neutrophilic) Leukopoiesis Maturation Medicine Medicine and Health Sciences Mice Mice, Inbred C57BL Molecular modelling Mutants Mutation Neutropenia Neutropenia - enzymology Neutropenia - genetics Neutrophils Neutrophils - cytology Neutrophils - enzymology Pathogenesis Peripheral blood Phosphatase Physiological aspects Protein folding Proteins Research and Analysis Methods Rodents Species Specificity Stem cell transplantation Stress Stresses Survival
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