Development and pathologies of the arterial wall

Cell Mol Life Sci. 2014 Jun;71(11):1977-99. doi: 10.1007/s00018-013-1478-y. Epub 2013 Sep 27.


Arteries consist of an inner single layer of endothelial cells surrounded by layers of smooth muscle and an outer adventitia. The majority of vascular developmental studies focus on the construction of endothelial networks through the process of angiogenesis. Although many devastating vascular diseases involve abnormalities in components of the smooth muscle and adventitia (i.e., the vascular wall), the morphogenesis of these layers has received relatively less attention. Here, we briefly review key elements underlying endothelial layer formation and then focus on vascular wall development, specifically on smooth muscle cell origins and differentiation, patterning of the vascular wall, and the role of extracellular matrix and adventitial progenitor cells. Finally, we discuss select human diseases characterized by marked vascular wall abnormalities. We propose that continuing to apply approaches from developmental biology to the study of vascular disease will stimulate important advancements in elucidating disease mechanism and devising novel therapeutic strategies.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Angiogenic Proteins / genetics
  • Angiogenic Proteins / metabolism
  • Animals
  • Arteries / growth & development
  • Arteries / metabolism
  • Arteries / pathology*
  • Cardiovascular Diseases / genetics
  • Cardiovascular Diseases / metabolism
  • Cardiovascular Diseases / pathology*
  • Cell Differentiation
  • Cell Lineage / genetics
  • Endothelial Cells / metabolism
  • Endothelial Cells / pathology
  • Endothelium, Vascular / growth & development
  • Endothelium, Vascular / metabolism
  • Endothelium, Vascular / pathology*
  • Gene Expression Regulation, Developmental
  • Humans
  • Morphogenesis / genetics*
  • Muscle, Smooth, Vascular / growth & development
  • Muscle, Smooth, Vascular / metabolism
  • Muscle, Smooth, Vascular / pathology*
  • Myocytes, Smooth Muscle / metabolism
  • Myocytes, Smooth Muscle / pathology
  • Neovascularization, Pathologic
  • Neovascularization, Physiologic


  • Angiogenic Proteins