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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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| Autores principales: | , , , , , , |
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| Formato: | Artigo |
| Lenguaje: | Inglês |
| Publicado: |
American Society of Hematology
2011
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| Materias: | |
| Acceso en línea: | 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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