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Article

Elastase inhibitors as potential therapies for ELANE-associated neutropenia.

Journal of leukocyte biology - 1 Oct 2017

Makaryan Vahagn, Kelley Merideth L, Fletcher Breanna, Bolyard Audrey Anna, Aprikyan A Andrew, Dale David C

Abstract excerpt

Mutations in ELANE, the gene for neutrophil elastase (NE), a protease expressed early in neutrophil development, are the most frequent cause of cyclic (CyN) and severe congenital neutropenia (SCN). We hypothesized that inhibitors of NE, acting either by directly inhibiting enzymatic activity or as chaperones for the mutant protein, might be effective as therapy for CyN and SCN. We investigated β-lactam-based...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 28754797
DOI: 10.1189/jlb.5A1016-445R
PMCID: PMC5597518

Genes

ELANE

Provenance

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Related research

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Elastase inhibitors as potential therapies for ELANE-associated neutropenia.PMID 28754797Read ↗

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Inducible expression of a disease-associated ELANE mutation impairs granulocytic differentiation, without eliciting an unfolded protein responseSemantic proximity Read ↗

CRISPR/Cas9-mediated ELANE knockout enables neutrophilic maturation of primary hematopoietic stem and progenitor cells and induced pluripotent stem cells of severe congenital neutropenia patientsSemantic proximity Read ↗

Four novel ELANE mutations in patients with congenital neutropenia.Semantic proximity Read ↗

CRISPR-Cas9-Mediated ELANE Mutation Correction in Hematopoietic Stem and Progenitor Cells to Treat Severe Congenital Neutropenia.Semantic proximity Read ↗

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Clinical characteristics of severe congenital neutropenia caused by novel ELANE gene mutations.Semantic proximity Read ↗

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