Characteristics of multi-organ lymphangiectasia resulting from temporal deletion of calcitonin receptor-like receptor in adult mice

PLoS One. 2012;7(9):e45261. doi: 10.1371/journal.pone.0045261. Epub 2012 Sep 17.

Abstract

Adrenomedullin (AM) and its receptor complexes, calcitonin receptor-like receptor (Calcrl) and receptor activity modifying protein 2/3, are highly expressed in lymphatic endothelial cells and are required for embryonic lymphatic development. To determine the role of Calcrl in adulthood, we used an inducible Cre-loxP system to temporally and ubiquitously delete Calcrl in adult mice. Following tamoxifen injection, Calcrl(fl/fl)/CAGGCre-ER™ mice rapidly developed corneal edema and inflammation that was preceded by and persistently associated with dilated corneoscleral lymphatics. Lacteals and submucosal lymphatic capillaries of the intestine were also dilated, while mesenteric collecting lymphatics failed to properly transport chyle after an acute Western Diet, culminating in chronic failure of Calcrl(fl/fl)/CAGGCre-ER™ mice to gain weight. Dermal lymphatic capillaries were also dilated and chronic edema challenge confirmed significant and prolonged dermal lymphatic insufficiency. In vivo and in vitro imaging of lymphatics with either genetic or pharmacologic inhibition of AM signaling revealed markedly disorganized lymphatic junctional proteins ZO-1 and VE-cadherin. The maintenance of AM signaling during adulthood is required for preserving normal lymphatic permeability and function. Collectively, these studies reveal a spectrum of lymphatic defects in adult Calcrl(fl/fl)/CAGGCre-ER™ mice that closely recapitulate the clinical symptoms of patients with corneal, intestinal and peripheral lymphangiectasia.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenomedullin / genetics
  • Adrenomedullin / metabolism
  • Animals
  • Antigens, CD / genetics
  • Antigens, CD / metabolism
  • Cadherins / genetics
  • Cadherins / metabolism
  • Calcitonin Receptor-Like Protein / deficiency
  • Calcitonin Receptor-Like Protein / genetics*
  • Edema / etiology
  • Edema / genetics*
  • Edema / metabolism
  • Edema / pathology
  • Endothelial Cells / drug effects
  • Endothelial Cells / metabolism
  • Endothelial Cells / pathology
  • Female
  • Gene Deletion
  • Gene Expression
  • Genetic Vectors
  • Intestinal Mucosa / metabolism
  • Intestines / drug effects
  • Intestines / pathology*
  • Limbus Corneae / drug effects
  • Limbus Corneae / metabolism
  • Limbus Corneae / pathology*
  • Lymphangiectasis / etiology
  • Lymphangiectasis / genetics*
  • Lymphangiectasis / metabolism
  • Lymphangiectasis / pathology
  • Lymphatic Vessels / drug effects
  • Lymphatic Vessels / metabolism
  • Lymphatic Vessels / pathology*
  • Male
  • Mice
  • Mice, Transgenic
  • Signal Transduction
  • Skin / drug effects
  • Skin / metabolism
  • Skin / pathology*
  • Tamoxifen / adverse effects
  • Zonula Occludens-1 Protein / genetics
  • Zonula Occludens-1 Protein / metabolism

Substances

  • Antigens, CD
  • Cadherins
  • Calcitonin Receptor-Like Protein
  • Calcrl protein, mouse
  • Tjp1 protein, mouse
  • Zonula Occludens-1 Protein
  • cadherin 5
  • Tamoxifen
  • Adrenomedullin