Incomplete splicing of neutrophil-specific genes affects neutrophil development in a zebrafish model of poikiloderma with neutropenia
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چکیده
منابع مشابه
A zebrafish model of Poikiloderma with Neutropenia recapitulates the human syndrome hallmarks and traces back neutropenia to the myeloid progenitor
Poikiloderma with Neutropenia (PN) is an autosomal recessive genodermatosis characterized by early-onset poikiloderma, pachyonychia, hyperkeratosis, bone anomalies and neutropenia, predisposing to myelodysplasia. The causative C16orf57/USB1 gene encodes a conserved phosphodiesterase that regulates the stability of spliceosomal U6-RNA. The involvement of USB1 in splicing has not yet allowed to u...
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15 صفحه اولAberrant expression of neutrophil and macrophage-related genes in a murine model for human neutrophil-specific granule deficiency.
Neutrophil-specific granule deficiency involves inheritance of germline mutations in the CCAAT/enhancer-binding protein epsilon (C/EBPE) gene. Humans and mice lacking active C/EBPepsilon suffer frequent bacterial infections as a result of functionally defective neutrophils and macrophages. We hypothesized that these defects reflected dysregulation of important immune response genes. To test thi...
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ژورنال
عنوان ژورنال: RNA Biology
سال: 2015
ISSN: 1547-6286,1555-8584
DOI: 10.1080/15476286.2015.1017240