Does fibrillin make elastin?
Does fibrillin make elastin?
In most tissues, fibrillin microfibrils associate with elastin to form elastic fibres and hence make key contributions to the elastic function of these tissues acting as a stiff reinforcer of elastin-containing tissues.
What does the fibrillin-1 gene do?
The FBN1 gene provides instructions for making a large protein called fibrillin-1. This protein is transported out of cells into the extracellular matrix, which is an intricate lattice of proteins and other molecules that forms in the spaces between cells.
What protein does fibrillin-1 affect?
Fibrillin-1 is a protein that in humans is encoded by the FBN1 gene, located on chromosome 15. It is a large, extracellular matrix glycoprotein that serves as a structural component of 10-12 nm calcium-binding microfibrils….Fibrillin-1.
| RNA expression pattern | |
|---|---|
| BioGPS | More reference expression data |
What is fibrillin in skin?
Fibrillin-1 is an important component of the dermal elastic fibre network, which fulfils key biomechanical and biochemical roles [17-19]. In the skin, fibrillin-1 is both a product of dermal fibroblasts and keratinocytes [20] and is deposited as an early event during the wound healing response [21].
How is elastin made?
Elastin is synthesized from the precursor molecule tropoelastin. Many molecules of tropoelastin are linked together to form the larger elastin complex. Each molecule of tropoelastin has 36 domains arranged in a random coil. It has alternating hydrophobic and hydrophilic domains encoded by separate regions of the gene.
What is fibrillin made of?
The structure of fibrillin consists of several cysteine-rich motifs and exhibits a multidomain organization similar to epidermal growth factor. The structure is stabilized by disulfide linkages. Fibrillin monomers undergo aggregation extracellularly into supramolecular structures.
How is Marfan syndrome diagnosed?
A blood test can be used to help diagnose Marfan syndrome. This blood test is highly specialized and looks for changes in FBN1, the gene that is responsible for most cases of Marfan syndrome. Genetic counseling should accompany genetic testing because FBN1 testing is not always straightforward.
What gene causes Marfan syndrome?
Marfan syndrome is caused by defects or deletions (mutations) of the fibrillin-1 (FBN1) gene.
What mutation causes Marfan syndrome?
Is fibrillin stretchy?
Fibrillin microfibrils, which have a beads-on-a-string appearance, are a major component of elastic fibres, conferring long range extensibility and contributing to the elastic deformation of tissues.
Is fibrillin a collagen?
Figure 1. Tendon, a complex structure made of collagen fibers constituted by collagen type I and pericellular matrix (PCM) mainly made of elastic fibers (EF): fibrillin 1 and 2, elastin, collagen type VI and others. Adapted from Grant et al. (2013).
What is elastin used for?
Elastin’s main function is to allow tissues in your body to stretch out and shrink back. Your arteries are tube-shaped blood vessels that carry blood from your heart through your body. Elastin gives your arteries stretchy characteristics that make it easier for your heart to pump blood.
