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Microfibrils and Fibrillin

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Book cover The Extracellular Matrix: an Overview

Part of the book series: Biology of Extracellular Matrix ((BEM))

Abstract

Microfibrils are supramolecular structures ubiquitously found in the extracellular matrix of elastic and nonelastic tissues. The three members of the cysteine-rich fibrillin family constitute the core of microfibrils. Mutations in fibrillin-1 and -2 lead to a number of heritable connective tissue disorders termed fibrillinopathies. Clinical symptoms affect blood vessels, bone, the eye, and other organ systems and highlight the importance of fibrillins in development and homeostasis of tissues and organs. Microfibrils have functional significance (1) in conferring mechanical stability and limited elasticity to tissues; (2) in the biogenesis and maintenance of the elastic fiber system; and (3) in the modulation of the activity of growth factors, including transforming growth factor-β and several bone morphogenetic proteins. In this chapter, we provide an overview of the structure, assembly, and functions of fibrillins and microfibrils and also the pathobiology associated with genetic aberrations in the microfibril system. Lessons learned from mouse models will be discussed as well as the emerging role of microfibrils and fibrillins in the regulation of growth factor bioavailability. Due to the large number of articles in the field, we repeatedly cite excellent review articles to which interested readers are referred to for more details.

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Abbreviations

BMP:

Bone morphogenetic protein

cbEGF:

Calcium-binding epidermal growth factor like domain

CCA:

Congenital contractural arachnodactyly

EGF:

Epidermal growth factor like domain

FBN1:

Human fibrillin-1 gene

Fbn1:

Mouse fibrillin-1 gene

FBN2:

Human fibrillin-2 gene

Fbn2:

Mouse fibrillin-2 gene

LTBP:

Latent transforming growth factor-β binding protein

TGF-β:

Transforming growth factor-β

Tsk:

Tight-skin mutation

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Acknowledgments

This work was supported by the Canadian Institutes of Health Research (MOP-68836 to DPR) and the German Academic Exchange Service DAAD (postdoctoral fellowship to DH).

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  1. Department of Anatomy and Cell Biology, Faculty of Medicine, McGill University Montreal, Montreal, QC, Canada, H3A 2B2

    Dirk Hubmacher & Dieter P. Reinhardt

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Correspondence to Dieter P. Reinhardt .

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  1. School of Medicine, Dept. Cell Biology & Physiology, Washington University, S. Euclid Avenue 660, St. Louis, 63110, Missouri, USA

    Robert P. Mecham

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Hubmacher, D., Reinhardt, D.P. (2011). Microfibrils and Fibrillin. In: Mecham, R. (eds) The Extracellular Matrix: an Overview. Biology of Extracellular Matrix. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-16555-9_7

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