PUBLICATION

Loss of Function of Parathyroid Hormone Receptor 1 Induces Notch-Dependent Aortic Defects During Zebrafish Vascular Development

Authors
Gray, C., Bratt, D., Lees, J., Dacosta, M., Plant, K., Solaymani-Kohal, S., Tazzyman, S., Serbanovic-Canic, J., Crossman, D.C., Keavney, B.D., Haase, A., McMahon, K., Gering, M., Roehl, H., Evans, P.C., and Chico, T.J.
ID
ZDB-PUB-130416-21
Date
2013
Source
Arterioscler. Thromb. Vasc. Biol.   33(6): 1257-63 (Journal)
Registered Authors
Chico, Tim J., Gering, Martin, Gray, Caroline, Roehl, Henry
Keywords
none
MeSH Terms
  • Animals
  • Aorta/embryology*
  • Aortic Coarctation/genetics
  • Aortic Coarctation/physiopathology
  • Female
  • Gene Expression Regulation, Developmental*
  • Homeodomain Proteins/genetics*
  • Male
  • Models, Animal
  • Mutation/genetics
  • Neovascularization, Physiologic/genetics
  • Nerve Tissue Proteins/genetics*
  • Receptor, Notch1/genetics*
  • Receptor, Parathyroid Hormone, Type 1/genetics*
  • Reference Values
  • Signal Transduction/genetics*
  • Up-Regulation
  • Zebrafish
  • Zebrafish Proteins/genetics*
PubMed
23559631 Full text @ Arterioscler. Thromb. Vasc. Biol.
Abstract

Objective—Coarctation of the aorta is rarely associated with known gene defects. Blomstrand chondrodysplasia, caused by mutations in the parathyroid hormone receptor 1 (PTHR1) is associated with coarctation of the aorta in some cases, although it is unclear whether PTHR1 deficiency causes coarctation of the aorta directly. The zebrafish allows the study of vascular development using approaches not possible in other models. We therefore examined the effect of loss of function of PTHR1 or its ligand parathyroid hormone-related peptide (PTHrP) on aortic formation in zebrafish.

Approach and Results—Morpholino antisense oligonucleotide knockdown of either PTHR1 or PTHrP led to a localized occlusion of the mid-aorta in developing zebrafish. Confocal imaging of transgenic embryos showed that these defects were caused by loss of endothelium, rather than failure to lumenize. Using a Notch reporter transgenic ([CSL:Venus]qmc61), we found both PTHR1 and PTHrP knockdown-induced defective Notch signaling in the hypochord at the site of the aortic defect before onset of circulation, and the aortic occlusion was rescued by inducible Notch upregulation.

Conclusions—Loss of function of either PTHR1 or PTHrP leads to a localized aortic defect that is Notch dependent. These findings may underlie the aortic defect seen in Blomstrand chondrodysplasia, and reveal a link between parathyroid hormone and Notch signaling during aortic development.

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