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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | IHC, ELISA, IP, ICC | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MAB2642 |
| Description | Anti-Fibrillin-2 Antibody, clone 48 |
| Alternate Names |
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| Background Information | Fibrillin-2 is a part of the Fibrillin family of glycoproteins, which are essential factors in the formation and maintenance of elastic fibers known as microfibrils within the extracellular matrix. Various researchers have suggested that Fibrillin-2 has a role in the mediation of microfibril assembly during early elastogenesis. Defects in Fibrillin-2 are causal to a rare disorder involving autosomal dominant connective tissue known as congenital contractual arachnodactyly (CCA), also known as Beals syndrome or distal arthrogryposis type 9 (DA9), and is similar to Marfan syndrome. |
| Product Information | |
|---|---|
| Format | Purified |
| Control |
|
| Presentation | Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Fibrillin-2 Antibody, clone 48 is an antibody against Fibrillin-2 for use in IH, ELISA, IP & IC. |
| Key Applications |
|
| Application Notes | ELISA Analysis: A previous lot was used by an independent laboratory in ELISA (Charbonneau, 2003). Immunofluorescence Analysis: A previous lot was used by an independent laboratory in IF (Charbonneau, 2003). |
| Biological Information | |
|---|---|
| Immunogen | Recombinant protein corresponding to human Fibrillin-2. |
| Epitope | Unknown |
| Clone | 48 |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Mouse |
| Specificity | The antibody recognizes Fibrillin-2. |
| Isotype | IgG1κ |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | The protein encoded by this gene is a component of connective tissue microfibrils and may be involved in elastic fiber assembly. Mutations in this gene cause congenital contractural arachnodactyly. [provided by RefSeq]. |
| Gene Symbol |
|
| Purification Method | Protein G Purified |
| UniProt Number |
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| UniProt Summary | FUNCTION: Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2-containing microfibrils regulate the early process of elastic fiber assembly. SUBCELLULAR LOCATION: Secreted › extracellular space › extracellular matrix. INVOLVEMENT IN DISEASE: Defects in FBN2 are the cause of congenital contractural arachnodactyly (CCA) [MIM:121050]; also known as Beals syndrome or distal arthrogryposis type 9 (DA9). CCA is a rare, autosomal dominant connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears. Phenotypically similar to Marfan syndrome, CCA does not affect the aorta and the eyes. SEQUENCE SIMILARITIES: Belongs to the fibrillin family. Contains 47 EGF-like domains. Contains 9 TB (TGF-beta binding) domains. |
| Molecular Weight | ~ 215 kDa |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Immunohistochemistry in liver cancer and papillary serous carcinoma paraffin-embedded tissue sections. Immunohistochemistry Analysis: 1:50 dilution of this antibody detected Fibrillin-2 in paraffin-embedded tissue sections of liver cancer and papillary serous carcinoma. |
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |