产品详情
简单介绍：
Anti-FBLN5 Antibody
详情介绍：
Overview
| Name: | Anti-FBLN5 Antibody See all FBLN5 primary antibodies
|
| Description: | Goat polyclonal antibody to FBLN5. |
| Applications: | ELISA, WB |
| Reactivity: | Human |
| Immunogen: | Synthetic peptide corresponding to Human FBLN5 (internal region). |
| Sequence: | C-RPIKGPREIQLDLE |
| Host: | Goat |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Unconjugated |
| Purification: | Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide. |
| Concentration: | 100 μg at 0.5 mg/ml. |
| Product Form: | Liquid |
| Formulation: | Supplied in Tris Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 0.5% BSA. |
| Storage: | Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles. |
Target
| Function: | Essential for elastic fiber formation, is involved in the assembly of continuous elastin (ELN) polymer and promotes the interaction of microfibrils and ELN (PubMed:18185537). Stabilizes and organizes elastic fibers in the skin, lung and vasculature (By similarity). Promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. Vascular ligand for integrin receptors which may play a role in vascular development and remodeling (PubMed:10428823). May act as an adapter that mediates the interaction between FBN1 and ELN (PubMed:17255108). |
| Tissue Specificity: | Expressed in skin fibroblasts (at protein level)(PubMed:17035250). Expressed predominantly in heart, ovary, and colon but also in kidney, pancreas, testis, lung and placenta. Not detectable in brain, liver, thymus, prostate, or peripheral blood leukocytes (PubMed:10428823). |
| Involvement in Disease: | Neuropathy, hereditary, with or without age-related macular degeneration: An autosomal dominant neuropathy of the Charcot-Marie-Tooth disease group, characterized by distal muscle weakness and atrophy variably affecting the lower and upper limbs. Distal sensory impairement and decreased nerve conduction velocities are present in most but not all patients. Additional variable features are age-related macular degeneration, joint hypermobility, and hyperelastic skin. Cutis laxa, autosomal dominant, 2: 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. Cutis laxa, autosomal recessive, 1A: 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. Macular degeneration, age-related, 3: A form of age-related macular degeneration, a multifactorial eye 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 that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. |
| Sequence Similarities: | Belongs to the fibulin family. |
| Post-Translational Modification: | N-glycosylated. |
| Cellular Location: | Secreted. Secreted > Extracellular space > Extracellular matrix. co-localizes with ELN in elastic fibers. |
| Database Links: |
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| Synonyms: | |
| Information: | Target information shown above is from the UniProt Consortium. |
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