ANG-1 TIE-2 and BMPR signalling defects are not seen in the nitrofen model of pulmonary hypertension and congenital diaphragmatic hernia.

Background: Pulmonary hypertension (PH) is a lethal disease that is associated with characteristic histological abnormalities of the lung vasculature and defects of angiopoetin-1 (ANG-1), TIE-2 and bone morphogenetic protein receptor (BMPR)-related signalling. We hypothesized that if these signalling defects cause PH generically, they will be readily identifiable perinatally in congenital diaphragmatic hernia (CDH), where the typical pulmonary vascular changes are present before birth and are accompanied by PH after birth.

Methods: CDH (predominantly left-sided, LCDH) was created in Sprague-Dawley rat pups by e9.

Neuropilins: a versatile partner of extracellular molecules that regulate development and disease.

Neuropilins are a vertebrate-specific family of membrane multidomain proteins. They are crucial for the embryonic development of neural and vascular systems, whereas in the adult organism they are implicated in many processes, such as angiogenesis and the immune response. Additionally, it has been shown that they are overexpressed in numerous types of tumours, which results in higher microvessel density and correlates with poor prognosis.

The heparanome and regulation of cell function: structures, functions and challenges.

The cell-extracellular matrix interface is a crowded space whose structure is dependent on macromolecular assemblies that are dynamic in time, molecular composition and location. Signals travel from one cell to another (or to the same cell) by the regulated assembly/disassembly of molecular complexes. These signals can evoke relatively simple biological responses such cell proliferation and migration, but once integrated, they guide cell fate in complex biological phenomena such as embryonic development and organism homeostasis.