Extract
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.
Abstract
Novel functions of the extracellular protein fibrillin-2 were discovered in tracheal tube development: fibrillin-2 has important roles in tracheal cartilage development and smooth muscle cell orientation and polarity, all required for tracheal contraction http://ow.ly/rn3Z30nId3N
Footnotes
Conflict of interest: H. Kumra has nothing to disclose.
Conflict of interest: N.E.H. Dinesh has nothing to disclose.
Conflict of interest: D.P. Reinhardt has nothing to disclose.
Support statement: Funding was received from the Canadian Institutes of Health Research and the Natural Sciences and Engineering Research Council of Canada. Funding information for this article has been deposited with the Crossref Funder Registry.
- Received January 18, 2019.
- Accepted February 9, 2019.
- Copyright ©ERS 2019