Evidence map›Paper›PMID 28811655›Full record

ArticleScientific reports2017

A Tissue Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome Using Human iPSC-derived Smooth Muscle Cells.

Leigh Atchison, Haoyue Zhang, Kan Cao, George A Truskey

Open access · goldAbstract read
In one paragraph

Article in Scientific reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 76 papers.

0numbers the graph read from it
0cells of the map it votes in
76citing papers in PubMed
4.9field-weighted citation impact, top 4% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

76 citing papers in PubMed, 111 citations in OpenAlex.

  1. Review
  2. Article
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  7. Microvascular Health as a Key Determinant of Organismal Aging.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025
    Review
  8. Article
  9. Review
  10. Article
  11. Review
  12. Article
  13. Article
  14. Article
  15. Use of iPSC-Derived Smooth Muscle Cells to Model Physiology and Pathology.Arteriosclerosis, thrombosis, and vascular biology · 2024
    Review
  16. Review
  17. Review
  18. Review
  19. Using Genomics to Identify Novel Therapeutic Targets for Aortic Disease.Arteriosclerosis, thrombosis, and vascular biology · 2024
    Review
  20. Review

16 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

4 authors at 2 institutions in 1 country.

Leigh AtchisonDepartment of Biomedical Engineering at Duke University, Durham, NC, 27708, United States.
Haoyue ZhangDepartment of Cell Biology and Molecular Genetics at University of Maryland, College Park, MD, 20742, United States.
Kan CaoDepartment of Cell Biology and Molecular Genetics at University of Maryland, College Park, MD, 20742, United States.
George A TruskeyDepartment of Biomedical Engineering at Duke University, Durham, NC, 27708, United States. george.truskey@duke.edu.
Duke University · USUniversity of Maryland, College Park · US

Funding

Preparation and Distribution of Adult Stem CellsP40OD011050 · OD · TEXAS A&M UNIVERSITY HEALTH SCIENCE CTR · PI PROCKOP, DARWIN JOHNSON · 2012 to 2017
$6.0M
Interaction of Subchondral Bone and Articular Cartilage System with Skeletal MuscleUH3TR000505 · NCATS · DUKE UNIVERSITY · PI TRUSKEY, GEORGE A · 2014 to 2016
$3.6M
Investigation of smooth muscle cell loss in progeriaR01HL126784 · NHLBI · UNIV OF MARYLAND, COLLEGE PARK · PI CAO, KAN · 2016 to 2020
$2.1M
NCATS NIH HHS UH3 TR000505NHLBI NIH HHS R01 HL126784NIH HHS P40 OD011050
6 · The paper itself

Abstract

Hutchison-Gilford Progeria Syndrome (HGPS) is a rare, accelerated aging disorder caused by nuclear accumulation of progerin, an altered form of the Lamin A gene. The primary cause of death is cardiovascular disease at about 14 years. Loss and dysfunction of smooth muscle cells (SMCs) in the vasculature may cause defects associated with HGPS. Due to limitations of 2D cell culture and mouse models, there is a need to develop improved models to discover novel therapeutics. To address this need, we produced a functional three-dimensional model of HGPS that replicates an arteriole-scale tissue engineered blood vessel (TEBV) using induced pluripotent stem cell (iPSC)-derived SMCs from an HGPS patient. To isolate the effect of the HGPS iSMCs, the endothelial layer consisted of human cord blood-derived endothelial progenitor cells (hCB-EPCs) from a separate, healthy donor. TEBVs fabricated from HGPS iSMCs and hCB-EPCs show reduced vasoactivity, increased medial wall thickness, increased calcification and apoptosis relative to TEBVs fabricated from normal iSMCs or primary MSCs. Additionally, treatment of HGPS TEBVs with the proposed therapeutic Everolimus, increases HGPS TEBV vasoactivity and increases iSMC differentiation in the TEBVs. These results show the ability of this iPSC-derived TEBV to reproduce key features of HGPS and respond to drugs.

Indexed as

Tissue EngineeringAnimalsBiomarkersBlood VesselsCell DifferentiationDisease Models, AnimalEverolimusFibroblastsGene ExpressionHumansInduced Pluripotent Stem CellsLamin Type AMutationMyocytes, Smooth MusclePhenotypeProgeriaBiomarkersEverolimusLamin Type ASirolimus

Identifiers

PMID28811655
PMCPMC5557922
OpenAlexW2745145572

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.