title: Vasculogenic and Angiogenic Pathways in Moyamoya Disease.
abstract: BACKGROUND
  Moyamoya disease (MMD) is a slowly progressing steno-occlusive cerebrovascular disease. The typical moyamoya vessels, which originate from an initial stenosis of the internal carotid, highlight that increased and/or abnormal angiogenic, vasculogenic and arteriogenic processes are involved in the disease pathophysiology.
  
  
  OBJECTIVE
  Herein, we summarize the current knowledge on the most important signaling pathways involved in MMD vessel formation, particularly focusing on the expression of growth factors and function of endothelial progenitor cells (EPCs).
  
  
  RESULTS
  Higher plasma concentrations of vascular endothelial growth factor, matrix metalloproteinase, hepatocyte growth factor, and interleukin-1β were reported in MMD. A specific higher level of basic fibroblast growth factor was also found in the cerebrospinal fluid of these patients. Finally, the number and the functionality of EPCs were found to be increased. In spite of the available data, the approaches and findings reported so far do not give an evident correlation between the expression levels of the aforementioned growth factors and MMD severity. Furthermore, the controversial results provided by studies on EPCs, do not permit to understand the true involvement of these cells in MMD pathophysiology.
  
  
  CONCLUSIONS
  Further studies should thus be implemented to extend our knowledge on processes regulating both the arterial stenosis and the excessive formation of collateral vessels. Moreover, we suggest advances of integrated approaches and functional assays to correlate biological and clinical data, arguing for the development of new therapeutic applications for MMD.
authors: Laboratory of Cellular Neurobiology, Neurology Unit, UCV, Neurological Institute "C. Besta" IRCCS Foundation, Via Celoria 11, Milan 20133, Italy. gloria.bedini@istituto-besta.it.
authors: Gloria
authors: G
authors: Bedini
authors: Kinga G
authors: KG
authors: Blecharz
authors: Sara
authors: S
authors: Nava
authors: Peter
authors: P
authors: Vajkoczy
authors: Giulio
authors: G
authors: Alessandri
authors: Michela
authors: M
authors: Ranieri
authors: Francesco
authors: F
authors: Acerbi
authors: Paolo
authors: P
authors: Ferroli
authors: Daria
authors: D
authors: Riva
authors: Silvia
authors: S
authors: Esposito
authors: Chiara
authors: C
authors: Pantaleoni
authors: Nardo
authors: N
authors: Nardocci
authors: Federica
authors: F
authors: Zibordi
authors: Elisa
authors: E
authors: Ciceri
authors: Eugenio A
authors: EA
authors: Parati
authors: Anna
authors: A
authors: Bersano
mesh_terms: D000818:Animals; D001808:Blood Vessels / Q000503:physiopathology*; D066026:Endothelial Progenitor Cells / Q000473:pathology; D006801:Humans; D018836:Inflammation Mediators / Q000378:metabolism; D009072:Moyamoya Disease / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D009389:Neovascularization, Pathologic
chemical_list: D018836:Inflammation Mediators