June 10, 2003BloodOpen Access

Analysis of intracellular storage and regulated secretion of 3 von Willebrand disease–causing variants of von Willebrand factor

View Full Paper

Ask AI

Bookmark

Population

HEK293 cells

Comparison

Transient transfection with 3 VWF mutations… vs Transient transfection with wild-type human VWF…

Design

Preclinical

Authors

GMGrégoire MichauxCentre National de la Recherche Scientifique LHLindsay HewlettNational Hospital for Neurology and Neurosurgery SMSarah L. MessengerUniversity of Sheffield

Discussion

Member takes

Implication

Links VWF mutation severity to WPB defects in models; extends mechanistic insights but leaves open human translation.

Structured PICO

Population

HEK293 cells

Intervention

Transient transfection with 3 VWF mutations causing von Willebrand disease

Comparator

Transient transfection with wild-type human VWF cDNA

Outcome

Formation, structure, and function (regulated secretion) of pseudo-Weibel-Palade bodies (WPBs)surrogate

VWF mutations causing von Willebrand disease impair the formation and morphology of endothelial storage organelles in proportion to clinical severity.

Cite This Study

Michaux et al. (2003) studied this question.

synapsesocial.com/papers/6a70ea5ba7fbea1e440840fc https://doi.org/10.1182/blood-2003-02-0599

View Full Paper

Ask AI

Bookmark

Analysis of intracellular storage and regulated secretion of 3 von Willebrand disease–causing variants of von Willebrand factor

Discussion

Member takes

Implication

Structured PICO

Cite This Study

Analysis of intracellular storage and regulated secretion of 3 von Willebrand disease–causing variants of von Willebrand factor

Discussion

Member takes

Implication

Structured PICO

Cite This Study

Also Consider

Also Consider