Severe Ankyrin-R deficiency results in impaired surface retention and lysosomal degradation of RhAG in human erythroblasts

Ankyrin-R provides a key link between band 3 and the spectrin cytoskeleton that helps to maintain the highly specialized erythrocyte biconcave shape. Ankyrin deficiency results in fragile spherocytic erythrocytes with reduced band 3 and protein 4.2 expression. We use in vitro differentiation of eryt...

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Publicado en:Haematologica
Main Authors: Satchwell, Timothy J., Bell, Amanda J., Hawley, Bethan R., Pellegrin, Stephanie, Mordue, Kathryn E., van Deursen, Cees Th. B. M., Braak, Nicole Heitink-ter, Huls, Gerwin, Leers, Mathie P.G, Overwater, Eline, Tamminga, Rienk Y. J., van der Zwaag, Bert, Fermo, Elisa, Bianchi, Paola, van Wijk, Richard, Toye, Ashley M.
Formato: Artigo
Idioma:Inglês
Publicado: Ferrata Storti Foundation 2016
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Articles
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC5060018/
https://ncbi.nlm.nih.gov/pubmed/27247322
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3324/haematol.2016.146209
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