Fibulin 3, Polyclonal Antibody

FBLN3, EGF-containing fibulin-like extracellular matrix protein 1, EFEMP1, DHRD, DRAD, FBNL, MLVT, MTLV, S1-5, FIBL-3

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Affinity chromatography purified via peptide column

Supplied in PBS containing 0.02% sodium azide.

Can be stored at -20 degree C, stable for one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Small volumes of anti-EFEMP1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Fibulin 3, also known as epidermal growth factor (EGF)-containing fibulin-like extracellular matrix protein 1 (EFEMP1), is a member of the fibulin family of extracellular glycoproteins, a group of proteins that are widely distributed and frequently associated with vascular and elastic tissues. The fibulin proteins typically contain a tandem array of EGF-like repeats and a fibulin-type COOH-terminal module. Aberrant accumulation of Fibulin 3 in the endoplasmic reticulum of retinal pigment epithelial cells has been shown to be associated with inherited forms of macular degeneration, but the loss of Fibulin 3 expression does not lead to macular degeneration but rather the appearance of hernias due to a reduction of elastic fibers of fascial connective tissue. Recent experiments have shown that expression of Fibulin 3 promotes tumor growth and may thus be a therapeutic target. At least three isoforms of Fibulin 3 are known to exist. This antibody is predicted to not cross-react with other Fibulin proteins.

Total protein Ab

ELISA (EIA), Western Blot (WB), Immunocytochemistry (ICC)

Western blot analysis of Fibulin 3 in HeLa cell lysate with Fibulin 3 antibody at (A) 0.5 and (B) 1 ug/mL.

Immunocytochemistry of Fibulin 3 in HeLa cells with Fibulin 3 antibody at 20 ug/mL.

39,195 Da[Similar Products]

EGF containing fibulin-like extracellular matrix protein 1

EGF-containing fibulin-like extracellular matrix protein 1

EGF-containing fibulin-like extracellular matrix protein 1

FBLN3; FBNL; FIBL-3  [Similar Products]

FBLN3_HUMAN

This gene encodes a member of the fibulin family of extracellular matrix glycoproteins. Like all members of this family, the encoded protein contains tandemly repeated epidermal growth factor-like repeats followed by a C-terminus fibulin-type domain. This gene is upregulated in malignant gliomas and may play a role in the aggressive nature of these tumors. Mutations in this gene are associated with Doyne honeycomb retinal dystrophy. Alternatively spliced transcript variants that encode the same protein have been described.[provided by RefSeq, Nov 2009]

EFEMP1: Binds EGFR, the EGF receptor, inducing EGFR autophosphorylation and the activation of downstream signaling pathways. May play a role in cell adhesion and migration. May function as a negative regulator of chondrocyte differentiation. In the olfactory epithelium, it may regulate glial cell migration, differentiation and the ability of glial cells to support neuronal neurite outgrowth. Defects in EFEMP1 are a cause of Doyne honeycomb retinal dystrophy (DHRD); also known as malattia leventinese (MLVT) (ML). DHRD is an autosomal dominant disease characterized by yellow-white deposits known as drusen that accumulate beneath the retinal pigment epithelium. Belongs to the fibulin family. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 2p16

Cellular Component: extracellular matrix; extracellular space; proteinaceous extracellular matrix; extracellular region

Molecular Function: protein binding; epidermal growth factor receptor activity; growth factor activity; calcium ion binding; epidermal growth factor receptor binding

Biological Process: epidermal growth factor receptor signaling pathway; extracellular matrix organization and biogenesis; peptidyl-tyrosine phosphorylation; visual perception; regulation of transcription, DNA-dependent; negative regulation of chondrocyte differentiation

Disease: Doyne Honeycomb Retinal Dystrophy

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