Activation of the unfolded protein response is associated with impaired granulopoiesis in transgenic mice expressing mutant Elane

Severe congenital neutropenia (SCN) is an inborn disorder of granulopoiesis that in many cases is caused by mutations of the ELANE gene, which encodes neutrophil elastase (NE). Recent data suggest a model in which ELANE mutations result in NE protein misfolding, induction of endoplasmic reticulum (E...

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Bibliografiske detaljer
Main Authors: Nanua, Suparna, Murakami, Mark, Xia, Jun, Grenda, David S., Woloszynek, Jill, Strand, Marie, Link, Daniel C.
Format: Artigo
Sprog:Inglês
Udgivet: American Society of Hematology 2011
Fag:
Hematopoiesis and Stem Cells
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3072877/
https://ncbi.nlm.nih.gov/pubmed/21285438
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood-2010-10-311704
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