VPS33B regulates protein sorting into and maturation of a-granule progenitor organelles in mouse megakaryocytes
نویسندگان
چکیده
Centre for Cardiovascular Sciences, School of Clinical and Experimental Medicine, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom; Medical Research Council, Laboratory for Molecular Cell Biology, University College London, London, United Kingdom; University College London Institute of Child Health, Gene Therapy Laboratory, London, United Kingdom; Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, Paris, France; Department of General Pediatrics, University Children’s Hospital, Muenster, Germany; Department of Pediatric Nephrology, University Hospital Vall d’Hebron, Barcelona, Spain; School of Cellular and Molecular Medicine, University of Bristol, Bristol, United Kingdom; and Inherited Metabolic Diseases, Great Ormond Street Hospital, London, United Kingdom
منابع مشابه
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, Vps33b(fl/fl)-ER(T2), and studied the platelet phenotype and α-granule biogenesis. Ultrastructural a...
متن کاملVPS33B regulates protein sorting into and maturation of α-granule progenitor organelles in mouse megakaryocytes Short title: VPS33B regulates α-granule progenitors’ maturation
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, Vps33b-ER, and studied the platelet phenotype and α-granule biogenesis. Ultrastructural analysis of V...
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Certain secretory proteins are known to be critical for maintaining the stemness of stem cells through autocrine signaling. However, the processes underlying the biogenesis, maturation, and secretion of these proteins remain largely unknown. Here we demonstrate that many secretory proteins produced by hematopoietic stem cells (HSCs) undergo exosomal maturation and release that is controlled by ...
متن کاملRequirement of VPS33B, a member of the Sec1/Munc18 protein family, in megakaryocyte and platelet -granule biogenesis
Bleeding problems are associated with defects in platelet -granules, yet little is known about how these granules are formed and released. Mutations affecting VPS33B, a novel Sec1/Munc18 protein, have recently been linked to arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome. We have characterized platelets from patients with ARC syndrome and observed reduced aggregation with ara...
متن کاملRequirement of VPS33B, a member of the Sec1/Munc18 protein family, in megakaryocyte and platelet alpha-granule biogenesis.
Bleeding problems are associated with defects in platelet alpha-granules, yet little is known about how these granules are formed and released. Mutations affecting VPS33B, a novel Sec1/Munc18 protein, have recently been linked to arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome. We have characterized platelets from patients with ARC syndrome and observed reduced aggregation wit...
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