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Aberrant subcellular targeting of the G185R neutrophil elastase mutant associated with severe congenital neutropenia induces premature apoptosis of differentiating promyelocytes

Mutations in the ELA2 gene encoding neutrophil elastase (NE) are present in most patients with severe congenital neutropenia (SCN). However, the mechanisms by which these mutations cause neutropenia remain unknown. To investigate the effects of mutant NE expression on granulopoiesis, we used the HL-...

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Bibliografiske detaljer
Main Authors: Massullo, Pam, Druhan, Lawrence J., Bunnell, Bruce A., Hunter, Melissa G., Robinson, John M., Marsh, Clay B., Avalos, Belinda R.
Format: Artigo
Sprog:Inglês
Udgivet: © 2005 by The American Society of Hematology 2005
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC1895019/
https://ncbi.nlm.nih.gov/pubmed/15657182
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood-2004-07-2618
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