FBLN5 Antibody (PACO17864)
제품 코드를 사용하여 Assay Genie 메인 사이트를 통해 주문합니다.
주문- SKU:
- PACO17864
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | FBLN5 Antibody (PACO17864) |
Antibody SKU: | PACO17864 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:50-1:200 |
Species Reactivity: | Human, Mouse, Rat |
Immunogen: | Synthetic peptide of human FBLN5 |
Form: | Liquid |
Storage Buffer: | -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
Purification Method: | Antigen affinity purification |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
The image on the left is immunohistochemistry of paraffin-embedded Human lung cancer tissue using PACO17864(FBLN5 Antibody) at dilution 1/85, on the right is treated with synthetic peptide. (Original magnification: x200). |
Background: | 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). |
Synonyms: | fibulin 5 |
UniProt Protein Function: | 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. |
UniProt Protein Details: | 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 |
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 Code: | Q9UBX5 |
NCBI GenInfo Identifier: | 12643876 |
NCBI Gene ID: | 10516 |
NCBI Accession: | Q9UBX5.1 |
UniProt Secondary Accession: | Q9UBX5,O75966, Q6IAL4, Q6UWA3, |
UniProt Related Accession: | Q9UBX5 |
Molecular Weight: | 50,180 Da |
NCBI Full Name: | Fibulin-5 |
NCBI Synonym Full Names: | fibulin 5 |
NCBI Official Symbol: | FBLN5 |
NCBI Official Synonym Symbols: | EVEC; UP50; ADCL2; ARMD3; DANCE; ARCL1A; FIBL-5; HNARMD |
NCBI Protein Information: | fibulin-5 |
UniProt Protein Name: | Fibulin-5 |
UniProt Synonym Protein Names: | Developmental arteries and neural crest EGF-like protein; Dance; Urine p50 protein; UP50 |
Protein Family: | Fibulin |
UniProt Gene Name: | FBLN5 |
UniProt Entry Name: | FBLN5_HUMAN |
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