FBLN1 is the gene encoding fibulin-1, an extracellular matrix and plasma protein.
Function
Fibulin-1 is a secreted
glycoprotein
Glycoproteins are proteins which contain oligosaccharide (sugar) chains covalently attached to amino acid side-chains. The carbohydrate is attached to the protein in a cotranslational or posttranslational modification. This process is known a ...
that is found in association with
extracellular matrix
In biology, the extracellular matrix (ECM), also called intercellular matrix (ICM), is a network consisting of extracellular macromolecules and minerals, such as collagen, enzymes, glycoproteins and hydroxyapatite that provide structural and bio ...
structures including
fibronectin
Fibronectin is a high- molecular weight (~500-~600 kDa) glycoprotein of the extracellular matrix that binds to membrane-spanning receptor proteins called integrins. Fibronectin also binds to other extracellular matrix proteins such as col ...
-containing fibers,
elastin
Elastin is a protein encoded by the ''ELN'' gene in humans and several other animals. Elastin is a key component in the extracellular matrix of gnathostomes (jawed vertebrates). It is highly Elasticity (physics), elastic and present in connective ...
-containing fibers and
basement membrane
The basement membrane, also known as base membrane, is a thin, pliable sheet-like type of extracellular matrix that provides cell and tissue support and acts as a platform for complex signalling. The basement membrane sits between epithelial tis ...
s. Fibulin-1 binds to a number of extracellular matrix constituents including
fibronectin
Fibronectin is a high- molecular weight (~500-~600 kDa) glycoprotein of the extracellular matrix that binds to membrane-spanning receptor proteins called integrins. Fibronectin also binds to other extracellular matrix proteins such as col ...
,
, and the
proteoglycan
Proteoglycans are proteins that are heavily glycosylated. The basic proteoglycan unit consists of a "core protein" with one or more covalently attached glycosaminoglycan (GAG) chain(s). The point of attachment is a serine (Ser) residue to w ...
,
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:
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NOV/CCN3,
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amyloid precursor protein
Amyloid-beta precursor protein (APP) is an integral membrane protein expressed in many biological tissue, tissues and concentrated in the synapses of neurons. It functions as a cell surface receptor and has been implicated as a regulator of s ...
,
*
entactin
Nidogen-1 (NID-1), formerly known as entactin, is a protein that in humans is encoded by the ''NID1'' gene. Both nidogen-1 and nidogen-2 are essential components of the basement membrane alongside other components such as type IV collagen, prote ...
,
*
fibrinogen,
and
*
fibronectin
Fibronectin is a high- molecular weight (~500-~600 kDa) glycoprotein of the extracellular matrix that binds to membrane-spanning receptor proteins called integrins. Fibronectin also binds to other extracellular matrix proteins such as col ...
.
See also
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Synpolydactyly
References
Further reading
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{{Calcium-binding proteins