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iPSC-Derived Endothelial Cells Affect Vascular Function in a Tissue-Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome.

Publication ,  Journal Article
Atchison, L; Abutaleb, NO; Snyder-Mounts, E; Gete, Y; Ladha, A; Ribar, T; Cao, K; Truskey, GA
Published in: Stem cell reports
February 2020

Hutchinson-Gilford progeria syndrome (HGPS) is a rare disorder caused by a point mutation in the Lamin A gene that produces the protein progerin. Progerin toxicity leads to accelerated aging and death from cardiovascular disease. To elucidate the effects of progerin on endothelial cells, we prepared tissue-engineered blood vessels (viTEBVs) using induced pluripotent stem cell-derived smooth muscle cells (viSMCs) and endothelial cells (viECs) from HGPS patients. HGPS viECs aligned with flow but exhibited reduced flow-responsive gene expression and altered NOS3 levels. Relative to viTEBVs with healthy cells, HGPS viTEBVs showed reduced function and exhibited markers of cardiovascular disease associated with endothelium. HGPS viTEBVs exhibited a reduction in both vasoconstriction and vasodilation. Preparing viTEBVs with HGPS viECs and healthy viSMCs only reduced vasodilation. Furthermore, HGPS viECs produced VCAM1 and E-selectin protein in TEBVs with healthy or HGPS viSMCs. In summary, the viTEBV model has identified a role of the endothelium in HGPS.

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Published In

Stem cell reports

DOI

EISSN

2213-6711

ISSN

2213-6711

Publication Date

February 2020

Volume

14

Issue

2

Start / End Page

325 / 337

Related Subject Headings

  • Tissue Engineering
  • Tissue Donors
  • Progeria
  • Phenotype
  • Models, Biological
  • Male
  • Induced Pluripotent Stem Cells
  • Humans
  • Gene Expression Regulation
  • Clone Cells
 

Citation

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ICMJE
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Atchison, L., Abutaleb, N. O., Snyder-Mounts, E., Gete, Y., Ladha, A., Ribar, T., … Truskey, G. A. (2020). iPSC-Derived Endothelial Cells Affect Vascular Function in a Tissue-Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome. Stem Cell Reports, 14(2), 325–337. https://doi.org/10.1016/j.stemcr.2020.01.005
Atchison, Leigh, Nadia O. Abutaleb, Elizabeth Snyder-Mounts, Yantenew Gete, Alim Ladha, Thomas Ribar, Kan Cao, and George A. Truskey. “iPSC-Derived Endothelial Cells Affect Vascular Function in a Tissue-Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome.Stem Cell Reports 14, no. 2 (February 2020): 325–37. https://doi.org/10.1016/j.stemcr.2020.01.005.
Atchison L, Abutaleb NO, Snyder-Mounts E, Gete Y, Ladha A, Ribar T, et al. iPSC-Derived Endothelial Cells Affect Vascular Function in a Tissue-Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome. Stem cell reports. 2020 Feb;14(2):325–37.
Atchison, Leigh, et al. “iPSC-Derived Endothelial Cells Affect Vascular Function in a Tissue-Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome.Stem Cell Reports, vol. 14, no. 2, Feb. 2020, pp. 325–37. Epmc, doi:10.1016/j.stemcr.2020.01.005.
Atchison L, Abutaleb NO, Snyder-Mounts E, Gete Y, Ladha A, Ribar T, Cao K, Truskey GA. iPSC-Derived Endothelial Cells Affect Vascular Function in a Tissue-Engineered Blood Vessel Model of Hutchinson-Gilford Progeria Syndrome. Stem cell reports. 2020 Feb;14(2):325–337.
Journal cover image

Published In

Stem cell reports

DOI

EISSN

2213-6711

ISSN

2213-6711

Publication Date

February 2020

Volume

14

Issue

2

Start / End Page

325 / 337

Related Subject Headings

  • Tissue Engineering
  • Tissue Donors
  • Progeria
  • Phenotype
  • Models, Biological
  • Male
  • Induced Pluripotent Stem Cells
  • Humans
  • Gene Expression Regulation
  • Clone Cells