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Article

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

Blood - 9 Jul 2015

Bem Danai, Smith Holly, Banushi Blerida, Burden Jemima J, White Ian J, Hanley Joanna, Jeremiah Nadia, Rieux-Laucat Frédéric, Bettels Ruth, Ariceta Gema, Mumford Andrew D, Thomas Steven G, Watson Steve P, Gissen Paul

Abstract excerpt

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 analysis of...

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Identifiers

PMID: 25947942
DOI: 10.1182/blood-2014-12-614677
PMCID: PMC4497959

Genes

ARC

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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