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 t...

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Detalles Bibliográficos
Autores principales: Colombo, Elisa A., Carra, Silvia, Fontana, Laura, Bresciani, Erica, Cotelli, Franco, Larizza, Lidia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2015
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4629135/
https://www.ncbi.nlm.nih.gov/pubmed/26522474
http://dx.doi.org/10.1038/srep15814

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