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Cellular Functions and Gene and Protein Expression Profiles in Endothelial Cells Derived from Moyamoya

Shuji Hamauchi1, Hideo Shichinohe1, Haruto Uchino1

  • 1Department of Neurosurgery, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

Plos One
|September 24, 2016
PubMed
Abstract

Insights

Moyamoya disease (MMD) endothelial cells (ECs) show impaired angiogenesis due to downregulated extracellular matrix (ECM) receptor genes. Splicing regulation proteins were upregulated in MMD ECs, indicating altered splicing patterns.

Area of Science:

  • Neuroscience
  • Vascular Biology
  • Stem Cell Biology

Background:

  • Moyamoya disease (MMD) is a progressive steno-occlusive cerebrovascular disorder.
  • The specific cellular and molecular characteristics of endothelial cells (ECs) in MMD remain largely uncharacterized.

Purpose of the Study:

  • To investigate the functional and molecular properties of endothelial cells (ECs) derived from induced pluripotent stem cells (iPSCs) of Moyamoya disease (MMD) patients.
  • To compare the proliferation, angiogenesis, and gene/protein expression profiles of MMD-derived ECs with those from healthy controls.

Main Methods:

  • Established iPSC lines from MMD patients (RNF213 R4810K variant) and healthy controls.
  • Differentiated iPSCs into ECs, purified using cell sorting (CD31+CD144+).
  • Assessed EC proliferation, angiogenesis, and response to angiogenic factors (VEGF, bFGF, TGF-β, BMP4). Performed DNA microarray and proteomics for comprehensive analysis.

Main Results:

  • Angiogenesis was significantly impaired in MMD-derived ECs, irrespective of angiogenic factors.
  • Endothelial proliferation rates were comparable between MMD and control ECs.
  • DNA microarray revealed downregulation of extracellular matrix (ECM) receptor-related genes (e.g., integrin β3) in MMD ECs.
  • Proteomics showed downregulation of cytoskeleton-related proteins and upregulation of splicing regulation-related proteins in MMD ECs.

Conclusions:

  • Downregulation of ECM receptor genes in MMD ECs may contribute to impaired angiogenic activity.
  • Upregulation of splicing regulation proteins suggests altered splicing patterns in MMD-derived ECs.
  • iPSC-derived ECs provide a valuable model for studying MMD pathogenesis at the cellular level.

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