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Deficiency of the ribosome biogenesis gene Sbds in hematopoietic stem and progenitor cells causes neutropenia in mice by attenuating lineage progression in myelocytes

Shwachman-Diamond syndrome is a congenital bone marrow failure disorder characterized by debilitating neutropenia. The disease is associated with loss-of-function mutations in the SBDS gene, implicated in ribosome biogenesis, but the cellular and molecular events driving cell specific phenotypes in...

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Detalhes bibliográficos
Publicado no:Haematologica
Main Authors: Zambetti, Noemi A., Bindels, Eric M. J., Van Strien, Paulina M. H., Valkhof, Marijke G., Adisty, Maria N., Hoogenboezem, Remco M., Sanders, Mathijs A., Rommens, Johanna M., Touw, Ivo P., Raaijmakers, Marc H. G. P.
Formato: Artigo
Idioma:Inglês
Publicado em: Ferrata Storti Foundation 2015
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4591760/
https://ncbi.nlm.nih.gov/pubmed/26185170
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3324/haematol.2015.131573
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