Lessons from tracheal tube development for understanding congenital tracheal malformations

Fibrillins constitute a family of extracellular proteins critical for the biogenesis of elastic fibres and for the activity regulation of growth factors of the transforming growth factor (TGF)-β superfamily. All three fibrillins are present during development of tissues and organs, including lung, aorta, bones and skin [1–3]. Typically, fibrillin-2 and -3 expression is limited to prenatal and early postnatal development in humans, whereas fibrillin-1 expression persists throughout adulthood. In mouse, the situation is simplified by the fact that fibrillin-3 is not expressed due to chromosomal rearrangement events [4]. In the developing mouse embryo, fibrillin-1 and -2 co-distribute in elastic and non-elastic tissues, with fibrillin-2 accumulating preferentially in elastic fibre-rich matrices [1]. Unlike fibrillin-1, the specific roles of fibrillin-2 (and fibrillin-3 in humans) in tissue development and homeostasis as well as in disease is still relatively little explored.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Editorials Source Type: research