Article
Severe Ankyrin-R deficiency results in impaired surface retention and lysosomal degradation of RhAG in human erythroblasts.
Haematologica - 1 Sept 2016
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
Abstract excerpt
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 erythroblasts transduced with shRNAs targeting ANK1 to generate erythroblasts and reticulocytes with a novel ankyrin-R...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.