fig1

Elastase-dependent congenital neutropenia

Figure 1. Outline of main steps leading to disruption of NE-mediated granulopoiesis. (A): Neutrophil development stages in the bone marrow, highlighting promyelocyte stage when differentiation blockade occurs. (B): ELANE frameshift mutations inducing cellular surveillance pathways that safeguard the quality of ELANE transcripts and prevent the production of mutant NE. (C): Mechanisms of mutant NE-induced neutropenia. The Figure was partly generated using Servier Medical Art, provided by Servier, licensed under a Creative Commons Attribution 3.0 unported license.

Rare Disease and Orphan Drugs Journal
ISSN 2771-2893 (Online)
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