Rabbit anti-Human FBLN5 Polyclonal Antibody | anti-FBLN5 antibody

FBLN5 Antibody

Cat. Num. AAA9402890

• Gene Names: FBLN5; EVEC; UP50; ADCL2; ARMD3; DANCE; ARCL1A; FIBL-5
• Reactivity: Human
• Applications: Immunohistochemistry
• Purity: Antigen affinity purification.
• Synonyms: FBLN5; Polyclonal Antibody; FBLN5 Antibody; EVEC; UP50; ADCL2; ARMD3; DANCE; ARCL1A; FIBL-5; anti-FBLN5 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous levels of total FBLN5 protein.
• Purity/Purification: Antigen affinity purification.
• Form/Format: Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
• Concentration: 3.1 mg/ml (varies by lot)
• Sequence Length: 448

• Applicable Applications for anti-FBLN5 antibody: Immunohistochemistry (IHC)
• Application Notes: Immunohistochemistry: 1:50-1:200
• Immunogen Type: Peptide
• Immunogen Description: Synthetic peptide corresponding to a region derived from internal residues of human fibulin 5
• Target Name: FBLN5
• Preparation and Storage: Store at -20 degree C

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded Human lung cancer tissue using at dilution 1/85.)

Related Product Information for anti-FBLN5 antibody

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).

Product Categories/Family for anti-FBLN5 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 19743803
• NCBI GeneID: 10516
• NCBI Accession #: NP_006320.2
• NCBI GenBank Nucleotide #: NM_006329.3
• UniProt Accession #: Q9UBX5; O75966; Q6IAL4; Q6UWA3
• Molecular Weight: 50,180 Da
• NCBI Official Full Name: fibulin-5
• NCBI Official Synonym Full Names: fibulin 5
• NCBI Official Symbol: FBLN5
• NCBI Official Synonym Symbols: EVEC; UP50; ADCL2; ARMD3; DANCE; ARCL1A; FIBL-5
• NCBI Protein Information: fibulin-5
• UniProt Protein Name: Fibulin-5
• Protein Family: Fibulin
• UniProt Gene Name: FBLN5
• UniProt Synonym Gene Names: DANCE; FIBL-5; Dance; UP50
• UniProt Entry Name: FBLN5_HUMAN

NCBI Description

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 Description

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, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 14q32.1

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

Molecular Function: protein C-terminus binding; integrin binding; protein binding; protein homodimerization activity; calcium ion binding

Biological Process: elastic fiber assembly; secretion; extracellular matrix organization and biogenesis; cell-matrix adhesion; regulation of cell growth

Disease: Cutis Laxa, Autosomal Dominant 2; Macular Degeneration, Age-related, 3; Cutis Laxa, Autosomal Recessive, Type Ia