Fibulin-1

FBLN1 is the gene encoding fibulin-1, an extracellular matrix and plasma protein.

Function

Fibulin-1 is a secreted glycoprotein that is found in association with extracellular matrix structures including fibronectin-containing fibers, elastin-containing fibers and basement membranes. Fibulin-1 binds to a number of extracellular matrix constituents including fibronectin, nidogen-1, and the proteoglycan, versican. Fibulin-1 is also a blood protein capable of binding to fibrinogen.

Structure

Fibulin-1 has modular domain structure and includes a series of nine epidermal growth factor-like modules followed by a fibulin-type module, a module found in all members of the fibulin gene family.

The human fibulin-1 gene, FBLN1, encodes four splice variants designated fibulin-1A, B, C and D, which differ in their carboxy terminal regions. In mouse, chicken and the nematode, '' C. elegans'', only two fibulin-1 variants are produced, fibulin-1C and fibulin-1D.

Interactions

FBLN1 has been shown to interact with:

* NOV/CCN3,
* amyloid precursor protein,
* entactin,
* fibrinogen, and
* fibronectin.

See also

* Synpolydactyly

References

Further reading

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{{Calcium-binding proteins