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Related Experiment Video

Updated: Jun 22, 2025

Microfluidic Model to Mimic Initial Event of Neovascularization

Microfluidic Model to Mimic Initial Event of Neovascularization

Published on: April 10, 2021

A Microphysiological HHT-on-a-Chip Platform Recapitulates Patient Vascular Lesions.

Christopher C W Hughes¹, Jennifer Fang², Christopher Hatch³

¹University of California, Irvine.

Research Square

|July 1, 2024

Summary

This summary is machine-generated.

This study introduces a novel HHT-on-a-chip model that replicates vascular malformations found in Hereditary Hemorrhagic Telangiectasia (HHT) patients. The model aids in understanding HHT disease and testing new drug therapies.

Keywords:

AVM Alk1 Biological Sciences Cell Biology HHT Hereditary Hemorrhagic Telangiectasia MPS systems arteriovenous malformation fluid shear stress microphysiological systems microvasculature organ-on-a-chip telangiectasia vascular malformation vessel-on-a-chip

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Last Updated: Jun 22, 2025

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Endothelialized Microfluidics for Studying Microvascular Interactions in Hematologic Diseases

Endothelialized Microfluidics for Studying Microvascular Interactions in Hematologic Diseases

Published on: June 22, 2012

Area of Science:

Vascular Biology
Rare Disease Research
Biomedical Engineering

Background:

Hereditary Hemorrhagic Telangiectasia (HHT) is a rare genetic disorder causing fragile vascular malformations (VMs).
Current understanding of HHT pathogenesis, particularly the role of ACVRL1 mutations, remains incomplete.
Limited treatment options exist for HHT patients.

Purpose of the Study:

To develop a human cell-based microphysiological model of HHT.
To investigate the biological mechanisms underlying HHT-related vascular malformations (VMs).
To identify potential therapeutic targets for HHT.

Main Methods:

Development of a Vascularized Micro-organ (VMO) platform for HHT modeling (HHT-VMO).
Utilized inducible ACVRL1-knockdown in primary human endothelial cells (EC) to control Alk1 expression.
Employed single-cell RNA sequencing and chimera experiments to analyze lesion formation.

Main Results:

The HHT-VMO model successfully recapitulated patient-specific VMs.
Evidence suggests cell non-autonomous effects and microvessel pruning in AVM formation.
VEGFR inhibitor pazopanib blocked lesion formation, aligning with clinical observations.

Conclusions:

The novel HHT-on-a-chip model provides a faithful platform for studying HHT disease.
The model facilitates deeper understanding of HHT pathophysiology, including the roles of Alk1 and PDGFB.
This platform can accelerate the discovery and testing of new HHT therapeutics.