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VPS33B regulates protein sorting into and maturation of α-granule progenitor organelles in mouse megakaryocytes

Arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome is caused by deficiencies in the trafficking proteins VPS33B or VIPAR, and is associated with a bleeding diathesis and a marked reduction in platelet α-granules. We generated a tamoxifen-inducible mouse model of VPS33B deficiency, Vps...

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Detalhes bibliográficos
Publicado no:Blood
Main Authors: Bem, Danai, Smith, Holly, Banushi, Blerida, Burden, Jemima J., White, Ian J., Hanley, Joanna, Jeremiah, Nadia, Rieux-Laucat, Frédéric, Bettels, Ruth, Ariceta, Gema, Mumford, Andrew D., Thomas, Steven G., Watson, Steve P., Gissen, Paul
Formato: Artigo
Idioma:Inglês
Publicado em: American Society of Hematology 2015
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4497959/
https://ncbi.nlm.nih.gov/pubmed/25947942
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1182/blood-2014-12-614677
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