Rabbit anti Human Fibulin-3
| Code | Size | Price |
|---|
| EXA-X2751P | 100 ug | £316.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Rabbit
Antibody Isotype: IgG
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Target Species: Human
Applications:
- Enzyme Immunoassay (EIA)
- Immunohistochemistry (IHC)
- Western Blot (WB)
Shipping:
Ship at ambient temperature freeze upon arrival
Storage:
Product should be stored at -20°C. Aliquot to avoid freeze/thaw cycles
Further Information
Applications Description:
Optimal concentration should be evaluated by serial dilutions.
Background:
Fibulin-1 is a modular glycoprotein component of elastic extracellular matrix fibers, basement membranes and blood. It can bind calcium, Fibronectin, Laminin, Nidogen and Fibrinogen. Estrogen exposure to ovarian cancer cell lines can upregulate Fibulin-1. Fibulin-2 is abundant in heart, placenta and ovarian tissue and binds several components of the extracelllar matrix including aggrecan, versican and brevican. Fibulin-3, also known as EFEMP1, is a secreted protein. Defects in the gene for Fibulin-3 cause the autosomal dominant disease Doyne honeycomb retinal dystrophy (DHRD, also known as malattia leventinese) which is characterized by yellow-white deposits (drusen) that accumulate under the retinal pigment epithelium. Fibulin-3 is not present at the site of drusen formation in normal eyes. Fibulin-5 is an integrin-binding extracellular matrix protein that mediates endothelial cell adhesion.
Caution:
This product is intended FOR RESEARCH USE ONLY, and FOR TESTS IN VITRO, not for use in diagnostic or therapeutic procedures involving humans or animals.
Concentration:
See vial for concentration
Field of Interest:
Cellular Structures
Formulation:
Provided as solution in phosphate buffered saline with 0.08% sodium azide
Functional Analysis:
Western Blotting
Immunogen:
Synthetic peptide derived from human fibulin-3 protein
Product Form:
Affinity Purified
Product Stability:
Products are stable for one year from purchase when stored properly
Purification Method:
Antigen Immunoaffiinity Purification
Synonyms:
EGF-containing fibulin-like extracellular matrix protein 1; Extracellular protein S1-5; Fibulin-like protein; EFEMP1
UniProt:
Q12805
References
1. Ikegawa, S., et al. Structure and chromosomal assignment of the human S1-5 gene (FBNL) that is highly homologous to fibrillin. Genomics 35: 590-592 (1996)
2. Stone, E.M., et al. A single EFEMP1 mutation associated with both malattia leventinese and Doyne honeycomb retinal dystrophy. Nat. Genet. 22: 199-202 (1999)
3. Giltay, R., et al. Sequence, recombinant expression and tissue localization of two novel extracellular matrix proteins, Fibulin-3 and Fibulin-4. Matrix Biol. 18: 469-480 (1999)
4. Matsumoto, M., et al. Dominant radial drusen and Arg345Trp EFEMP1 mutation. Am. J. Ophthalmol. 131: 810-812 (2001)
5. Marmorstein, L.Y., et al. Aberrant accumulation of EFEMP1 underlies drusen formation in malattia leventinese and age-related macular degeneration. Proc. Natl. Acad. Sci. USA 99: 13067-13072 (2002)
6. Marmorstein, L.Y., et al. Association of EFEMP1 with malattia leventinese and age-related macular degeneration: a mini-review. Ophthalmic Genet. 25: 219-226 (2004)
7. Klenotic, P.A., et al. Tissue inhibitor of metalloproteinases-3 (TIMP-3) is a binding partner of epithelial growth factor-containing fibulin-like extracellular matrix protein 1 EFEMP1). Implications for macular degenerations. J. Biol. Chem. 279: 30469-30473 (2004)
8. Narendran, N., et al. Analysis of the EFEMP1 gene in individuals and families with early onset drusen. Eye 19: 11-15 (2005)
2. Stone, E.M., et al. A single EFEMP1 mutation associated with both malattia leventinese and Doyne honeycomb retinal dystrophy. Nat. Genet. 22: 199-202 (1999)
3. Giltay, R., et al. Sequence, recombinant expression and tissue localization of two novel extracellular matrix proteins, Fibulin-3 and Fibulin-4. Matrix Biol. 18: 469-480 (1999)
4. Matsumoto, M., et al. Dominant radial drusen and Arg345Trp EFEMP1 mutation. Am. J. Ophthalmol. 131: 810-812 (2001)
5. Marmorstein, L.Y., et al. Aberrant accumulation of EFEMP1 underlies drusen formation in malattia leventinese and age-related macular degeneration. Proc. Natl. Acad. Sci. USA 99: 13067-13072 (2002)
6. Marmorstein, L.Y., et al. Association of EFEMP1 with malattia leventinese and age-related macular degeneration: a mini-review. Ophthalmic Genet. 25: 219-226 (2004)
7. Klenotic, P.A., et al. Tissue inhibitor of metalloproteinases-3 (TIMP-3) is a binding partner of epithelial growth factor-containing fibulin-like extracellular matrix protein 1 EFEMP1). Implications for macular degenerations. J. Biol. Chem. 279: 30469-30473 (2004)
8. Narendran, N., et al. Analysis of the EFEMP1 gene in individuals and families with early onset drusen. Eye 19: 11-15 (2005)