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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| B, Ch, H, M, R | IF, WB, ICC | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | CBL173 |
| Brand Family | Chemicon® |
| Trade Name |
|
| Description | Anti-Desmoplakin 1& 2 Antibody, clone DP2.15 |
| Product Information | |
|---|---|
| Format | Purified |
| Presentation | Lyophilized. Reconstitute in 1 mL distilled water (final solution contains 0.09% sodium azide, 0.5% BSA in PBS buffer, pH 7.4 |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Desmoplakin 1& 2 Antibody, clone DP2.15 detects level of Desmoplakin 1& 2 & has been published & validated for use in IF, WB & IC. |
| Key Applications |
|
| Application Notes | Western blot Immunohistochemistry: 1:10; Frozen tissue Immunofluorescence Optimal working dilutions must be determined by the end user. |
| Biological Information | |
|---|---|
| Immunogen | Bovine desmoplakin 1 & 2 |
| Clone | DP2.15 |
| Host | Mouse |
| Specificity | This antibody shows distinct punctate membrane staining of desmoplakins 1 (MW 250 kDa) and 2 (MW 215 kDa) in stratified epithelia and simple epithelia, including glands, urothelium, thymic reticular epithelium, hepatocytes, intercalated disks of myocardium and arachnoid cells of meninges. |
| Isotype | IgG1 |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | Desmosomes are intercellular junctions that tightly link adjacent cells. Desmoplakin is an obligate component of functional desmosomes that anchors intermediate filaments to desmosomal plaques. The N-terminus of desmoplakin is required for localization to the desmosome and interacts with the N-terminal region of plakophilin 1 and plakoglobin. The C-terminus of desmoplakin binds with intermediate filaments. In the mid-region of desmoplakin, a coiled-coiled rod domain is responsible for homodimerization. Mutations in this gene are the cause of several cardiomyopathies and keratodermas as well as the autoimmune disease paraneoplastic pemphigus. |
| Gene Symbol |
|
| UniProt Number |
|
| UniProt Summary | FUNCTION: SwissProt: P15924 # Major high molecular weight protein of desmosomes. Involved in the organization of the desmosomal cadherin- plakoglobin complexes into discrete plasma membrane domains and in the anchoring of intermediate filaments to the desmosomes. SIZE: 2871 amino acids; 331774 Da SUBUNIT: Homodimer. SUBCELLULAR LOCATION: Note=Innermost portion of the desmosomal plaque. TISSUE SPECIFICITY: Isoform DPI is apparently an obligate constituent of all desmosomes; Isoform DPII resides predominantly in tissues and cells of stratified origin. DOMAIN: SwissProt: P15924 The N-terminal region is required for localization to the desmosomal plaque and interacts with the N-terminal region of plakophilin 1. The C-terminal region interacts with intermediate filaments. PTM: Substrate of transglutaminase. Some glutamines and lysines are cross-linked to other desmoplakin molecules, to other proteins such as keratin, envoplakin, periplakin and involucrin, and to lipids like omega-hydroxyceramide. DISEASE: "SwissProt: P15924 # Defects in DSP are the cause of keratosis palmoplantaris striata II (PPKS2) [MIM:125647]; also known as striate palmoplantar keratoderma II (SPPK2). PPKS2 is characterized by skin thickening in the palms (linear pattern) and the soles (island-like pattern) and flexor aspect of the fingers; and rarely by abnormalities of the nails, the teeth and the hair. & Defects in DSP are the cause of dilated cardiomyopathy with woolly hair and keratoderma (DCWHK) [MIM:605676]; also known as Carvajal syndrome or palmoplantar keratoderma with left ventricular cardiomyopathy and woolly hair. DCWHK is an autosomal recessive cardiocutaneous syndrome characterized by a generalized striate keratoderma particularly affecting the palmoplantar epidermis, woolly hair, and dilated left ventricular cardiomyopathy. & Defects in DSP are the cause of familial arrhythmogenic right ventricular dysplasia 8 (ARVD8) [MIM:607450]; also known as arrhythmogenic right ventricular cardiomyopathy 8 (ARVC8). ARVD is an autosomal dominant disease characterized by partial degeneration of the myocardium of the right ventricle, electrical instability, and sudden death. It is clinically defined by electrocardiographic and angiographic criteria; pathologic findings, replacement of ventricular myocardium with fatty and fibrous elements, preferentially involve the right ventricular free wall. & Defects in DSP are the cause of skin fragility-woolly hair syndrome (SFWHS) [MIM:607655]. SFWHS is an autosomal recessive genodermatosis characterized by focal and diffuse palmoplantar keratoderma, hyperkeratotic plaques on the trunk and limbs, and woolly hair with varying degrees of alopecia. & Defects in DSP are the cause of lethal acantholytic epidermolysis bullosa [MIM:609638]. Lethal acantholytic epidermolysis bullosa is characterized by severe fragility of skin and mucous membranes. The phenotype is lethal in the neonatal period because of immense transcutaneous fluid loss. Typical features include universal alopecia, neonatal teeth, and nail loss. Histopathology of the skin shows suprabasal clefting and acantholysis throughout the spinous layer, mimicking pemphigus." SIMILARITY: Belongs to the plakin or cytolinker family. & Contains 17 plectin repeats. & Contains 2 spectrin repeats. |
| Product Usage Statements | |
|---|---|
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Reconstituted antibody can be stored at 2°-8°C for up to 12 months from date of receipt. Antibody can also be stored at -20°C in undiluted aliquots. |
| Packaging Information | |
|---|---|
| Material Size | 50 µg |