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

Author:

Bem Danai1,Smith Holly12,Banushi Blerida2,Burden Jemima J.2,White Ian J.2,Hanley Joanna23,Jeremiah Nadia4,Rieux-Laucat Frédéric4,Bettels Ruth5,Ariceta Gema6,Mumford Andrew D.7,Thomas Steven G.1,Watson Steve P.1,Gissen Paul28

Affiliation:

1. Centre for Cardiovascular Sciences, School of Clinical and Experimental Medicine, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom;

2. Medical Research Council, Laboratory for Molecular Cell Biology, University College London, London, United Kingdom;

3. University College London Institute of Child Health, Gene Therapy Laboratory, London, United Kingdom;

4. Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, Paris, France;

5. Department of General Pediatrics, University Children’s Hospital, Muenster, Germany;

6. Department of Pediatric Nephrology, University Hospital Vall d’Hebron, Barcelona, Spain;

7. School of Cellular and Molecular Medicine, University of Bristol, Bristol, United Kingdom; and

8. Inherited Metabolic Diseases, Great Ormond Street Hospital, London, United Kingdom

Abstract

Key PointsHematologic effects in the mouse model for ARC syndrome, Vps33bfl/fl-ERT2, in which Vps33b is ubiquitously excised post-development. The VPS33B-VIPAR complex is responsible for sorting cargo to and maturation of α-granule-destined MVBs.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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