货号：AB10400

品牌：Merck Millipore

规格：EA

目录价：询价

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Desmoplakin 1 Antibody | AB10400 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Background Information: Desmoplakin is a cytoplasmic plaque protein that is located in desmosomes, which are abundant in tissues subjected to mechanical stress, like muscle and epidermis. The role of Desmoplakin is to link the transmembrane cadherins via plakoglobin to the cytoplasmic intermediate filament network. Desmoplakin comprises an N-terminal, desmosome-associated plakin domain binding to plakoglobin and plakophilin, a central coiled-coil rod domain responsible for dimerization, and a C-terminal domain consisting of three homologous plakin repeat subdomains, named A, B, and C, that interact with the intermediate filaments View All» Species Reactivity: Human View All» Application Notes: Suggested dilutions: Western Blot: 1:5000 View All» Purification Method: Immunoaffinity purified View All» Presentation: Liquid containing 0.02M PBS containing 0.1% sodium azide and 50% glycerol View All» Storage Conditions: 1 year at 4°C from date of shipment View All» UniProt Number: P15924 View All» Entrez Gene Number:

• NM_001008844.1

• NM_004415.2
  View All» Gene Symbol:
  • DSP

  • DPI

  • Desmoplakin

  • PPKS2

  • KPPS2

  • DP

  • DPII
    View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications: Western Blotting View All» 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. View All» 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. View All» Brand Family: Chemicon View All» Product Name: Anti-Desmoplakin 1 Antibody | AB10400 View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 100 µL View All» Format: Affinity Purified View All» Host: Rabbit View All»
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