ArticleBlood2010
Hereditary spherocytosis and hereditary elliptocytosis: aberrant protein sorting during erythroblast enucleation.
Article in Blood, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed, 56 citations in OpenAlex.
- New insights into the mechanisms of red blood cell enucleation: From basics to clinical applications.EJHaem · 2024Review
- Erythroblast enucleation at a glance.Journal of cell science · 2024Review
- Splenectomy improves erythrocyte functionality in spherocytosis based on septin abundance, but not maturation defects.Blood advances · 2023Article
- Aberrant Membrane Composition and Biophysical Properties Impair Erythrocyte Morphology and Functionality in Elliptocytosis.Biomolecules · 2020Article
- Expression of South East Asian Ovalocytic Band 3 Disrupts Erythroblast Cytokinesis and Reticulocyte Maturation.Frontiers in physiology · 2020Article
- Cellular dynamics of mammalian red blood cell production in the erythroblastic island niche.Biophysical reviews · 2019Review
- Glycophorin-C sialylation regulates Lu/BCAM adhesive capacity during erythrocyte aging.Blood advances · 2018Article
- The Shape Shifting Story of Reticulocyte Maturation.Frontiers in physiology · 2018Review
- 'Gardos Channelopathy': a variant of hereditary Stomatocytosis with complex molecular regulation.Scientific reports · 2017Article
- Hereditary Xerocytosis due to Mutations inCase reports in hematology · 2017Article
- From the Cradle to the Grave: The Role of Macrophages in Erythropoiesis and Erythrophagocytosis.Frontiers in immunology · 2017Review
- Diffusion of glycophorin A in human erythrocytes.Biochimica et biophysica acta · 2016Article
- Severe Ankyrin-R deficiency results in impaired surface retention and lysosomal degradation of RhAG in human erythroblasts.Haematologica · 2016Article
- The human Kell blood group binds the erythroid 4.1R protein: new insights into the 4.1R-dependent red cell membrane complex.British journal of haematology · 2015Article
- The sorting of blood group active proteins during enucleation.ISBT science series · 2015Article
- A 130-kDa protein 4.1B regulates cell adhesion, spreading, and migration of mouse embryo fibroblasts by influencing actin cytoskeleton organization.The Journal of biological chemistry · 2014Article
- Mice expressing RHAG and RHD human blood group genes.PloS one · 2013Article
- Murine bone marrow Lin⁻Sca⁻1⁺CD45⁻ very small embryonic-like (VSEL) cells are heterogeneous population lacking Oct-4A expression.PloS one · 2013Article
- Article
- Brain, blood, and iron: perspectives on the roles of erythrocytes and iron in neurodegeneration.Neurobiology of disease · 2012Review
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6 authors at 2 institutions in 1 country.
Funding
Abstract
During erythroblast enucleation, membrane proteins distribute between extruded nuclei and reticulocytes. In hereditary spherocytosis (HS) and hereditary elliptocytosis (HE), deficiencies of membrane proteins, in addition to those encoded by the mutant gene, occur. Elliptocytes, resulting from protein 4.1R gene mutations, lack not only 4.1R but also glycophorin C, which links the cytoskeleton and bilayer. In HS resulting from ankyrin-1 mutations, band 3, Rh-associated antigen, and glycophorin A are deficient. The current study was undertaken to explore whether aberrant protein sorting, during enucleation, creates these membrane-spanning protein deficiencies. We found that although glycophorin C sorts to reticulocytes normally, it distributes to nuclei in 4.1R-deficient HE cells. Further, glycophorin A and Rh-associated antigen, which normally partition predominantly to reticulocytes, distribute to both nuclei and reticulocytes in an ankyrin-1-deficient murine model of HS. We conclude that aberrant protein sorting is one mechanistic basis for protein deficiencies in HE and HS.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.