Goat anti-Fibulin 5/FBLN5 Antibody | MyBioSource MBS421502 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Goat anti-Fibulin 5/FBLN5 Antibody

catalog :

MBS421502

quantity :

0.1 mg

price :

300 USD

clonality :

polyclonal

host :

goat

conjugate :

nonconjugated

reactivity :

human, mouse, rat, dog

application :

western blot, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS421502

products type :

Antibody

products full name :

Goat anti-Fibulin 5/FBLN5 Antibody

products short name :

Fibulin 5/FBLN5

products name syn :

FBLN5; fibulin 5; ARMD3; DANCE; EVEC; FLJ90059; UP50; developmental arteries and neural crest epidermal growth factor-like; urine p50 protein; FBLN5 antibody; fibulin 5 antibody; ARMD3 antibody; DANCE antibody; EVEC antibody; FLJ90059 antibody; UP50 antibody; developmental arteries and neural crest epidermal growth factor-like antibody; urine p50 protein antibody; Fibulin 5; Fibulin 5 / FBLN5

other names :

fibulin-5; Fibulin-5; fibulin-5; fibulin 5; Developmental arteries and neural crest EGF-like protein; Dance; Urine p50 protein; UP50

products gene name :

FBLN5

other gene names :

FBLN5; FBLN5; EVEC; UP50; ADCL2; ARMD3; DANCE; ARCL1A; FIBL-5; HNARMD; DANCE; FIBL-5; Dance; UP50

uniprot entry name :

FBLN5_HUMAN

clonality :

Polyclonal

host :

Goat

reactivity :

Tested: Human; Expected from sequence similarity: Human, Mouse, Rat, Dog

sequence length :

448

sequence :

RPIKGPREIQLDLE

purity :

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide

form :

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

concentration :

100ug specific antibody in 200ul

storage stability :

Aliquot and store at -20 degree C. Minimize freezing and thawing.

tested application :

Peptide ELISA (EIA), Western Blot (WB)

app notes :

Peptide ELISA: Antibody detection limit dilution 1: 32000.

other info1 :

Immunogen: Peptide with sequence C-RPIKGPREIQLDLE, from the internal region (near the C Terminus) of the protein sequence according to NP_006320.2. Epitope: Internal region (near the C Terminus)

ncbi gi num :

19743803

ncbi acc num :

NP_006320.2

ncbi gb acc num :

NM_006329.3

ncbi mol weight :

50,180 Da

ncbi pathways :

Elastic Fibre Formation Pathway (1270251); Extracellular Matrix Organization Pathway (1270244); Molecules Associated With Elastic Fibres Pathway (1270252)

ncbi summary :

The protein encoded by this gene is a secreted, extracellular matrix protein containing an Arg-Gly-Asp (RGD) motif and calcium-binding EGF-like domains. It promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. It is prominently expressed in developing arteries but less so in adult vessels. However, its expression is reinduced in balloon-injured vessels and atherosclerotic lesions, notably in intimal vascular smooth muscle cells and endothelial cells. Therefore, the protein encoded by this gene may play a role in vascular development and remodeling. Defects in this gene are a cause of autosomal dominant cutis laxa, autosomal recessive cutis laxa type I (CL type I), and age-related macular degeneration type 3 (ARMD3). [provided by RefSeq, Jul 2008]

uniprot summary :

FBLN5: Promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. Could be a vascular ligand for integrin receptors and may play a role in vascular development and remodeling. Defects in FBLN5 are the cause of cutis laxa, autosomal dominant, type 2 (ADCL2). A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. Additional variable clinical features are gastrointestinal diverticula, hernia, and genital prolapse. Rare manifestations are pulmonary artery stenosis, aortic aneurysm, bronchiectasis, and emphysema. Defects in FBLN5 are a cause of cutis laxa, autosomal recessive, type 1A (ARCL1A). A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. The clinical spectrum of autosomal recessive cutis laxa is highly heterogeneous with respect to organ involvement and severity. Type I autosomal recessive cutis laxa is a specific, life-threatening disorder with organ involvement, lung atelectasis and emphysema, diverticula of the gastrointestinal and genitourinary systems, and vascular anomalies. Associated cranial anomalies, late closure of the fontanel, joint laxity, hip dislocation, and inguinal hernia have been observed but are uncommon. Defects in FBLN5 are the cause of age-related macular degeneration type 3 (ARMD3). ARMD is a multifactorial disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Belongs to the fibulin family. Protein type: Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 14q32.1. Cellular Component: extracellular matrix; extracellular region; extracellular space; proteinaceous extracellular matrix. Molecular Function: calcium ion binding; integrin binding; protein binding; protein C-terminus binding; protein homodimerization activity. Biological Process: cell-matrix adhesion; elastic fiber assembly; extracellular matrix organization and biogenesis; regulation of cell growth; secretion. Disease: Cutis Laxa, Autosomal Dominant 2; Cutis Laxa, Autosomal Recessive, Type Ia; Macular Degeneration, Age-related, 3

size1 :

0.1 mg

price1 :

300 USD

more info or order :

MyBioSource product webpage