FITC标记的桥粒斑蛋白1抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的桥粒斑蛋白1抗体

FITC标记的桥粒斑蛋白1抗体

商家询价

产品名称： FITC标记的桥粒斑蛋白1抗体

英文名称： Anti-Desmoplakin I+II/FITC

产品编号： HZ-1748R-FITC

产品价格： null

产品产地： 中国/上海

品牌商标： HZbscience

更新时间： 2023-08-17T10:24:20

使用范围： IF=1:50-200

上海沪震实业有限公司
  • 联系人 : 鲍丽雯
  • 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
  • 邮编 : 200612
  • 所在区域 : 上海
  • 电话 : 139****0749 点击查看
  • 传真 : 点击查看
  • 邮箱 : www.shzbio.net
  • 二维码 : 点击查看
进入展台

 Rabbit Anti-Desmoplakin I+II/FITC Conjugated antibody

FITC标记的桥粒斑蛋白1抗体

 

英文名称 Anti-Desmoplakin I+II/FITC
中文名称 FITC标记的桥粒斑蛋白1抗体
别    名 Desmoplakin I; 250/210 kDa paraneoplastic pemphigus antigen; Desmoplakin (DPI DPII); Desmoplakin; Desmoplakin II; DP; DP I; DP II; DPI; DP-I; DPII; DSP; PPKS 2; PPKS2; DESP_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Horse, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 333kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DP-I
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
Desmosomes are intercellular junctions that form tight links between adjacent cells. Desmoplakin is an obligate component of functional desmosomes that attaches intermediate filaments to desmosomal plaques. It is involved in the organization of desmosomal cadherin-plakoglobin complexes into discrete plasma membrane domains. The N-terminus of desmoplakin is essential for localisation to the desmosome and interaction with plakophilin 1 and plakoglobin. The C-terminus of desmoplakin binds to intermediate filaments. The central region of desmoplakin comprises a coiled-coil rod domain that mediates homodimerisation. There are two isoforms of desmoplakin - desmoplakin I, which is an obligate component of all desmosomes, and desmoplakin II, which is predominantly expressed in tissues and cells of stratified origin. Mutations in the gene encoding desmoplakin result in a number of cardiomyopathies and keratodermas as well as the autoimmune disease paraneoplastic pemphigus.

Function:
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.

Subunit:
Homodimer. Interacts with COL17A1 (via cytoplasmic region). Associates (via C-terminal) with KRT5-KRT14 (via rod region), KRT8-KRT18 and VIM intermediate filaments. Interacts with DSC2.

Subcellular Location:
Cell junction, desmosome. Cytoplasm, cytoskeleton. Note=Innermost portion of the desmosomal plaque. Colocalizes with epidermal KRT5-KRT14 and simple KRT8-KRT18 keratins and VIM intermediate filaments network.

Tissue Specificity:
Isoform DPI is apparently an obligate constituent of all desmosomes. Isoform DPII resides predominantly in tissues and cells of stratified origin.

Post-translational modifications:
Ser-2849 is probably phosphorylated by a cAMP-dependent protein kinase. Phosphorylation on Ser-2849 probably affects its association with epidermal, simple cytokeratins and VIM intermediate filaments.
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:
Defects in DSP are the cause of palmoplantar keratoderma striate type 2 (SPPK2) [MIM:612908]; also known as keratosis palmoplantaris striata II. SPPK2 is characterized by skin thickening in the palms (linear pattern) and the soles (island-like pattern) and flexor aspect of the fingers. Abnormalities of the nails, the teeth and the hair are rarely present. 
Defects in DSP are the cause of cardiomyopathy dilated 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 
Defects in DSP are the cause of familial arrhythmogenic right ventricular dysplasia type 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 epidermolysis bullosa lethal acantholytic (EBLA) [MIM:609638]. EBLA 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 1 SH3 domain. 
Contains 6 spectrin repeats.

Database links:

Entrez Gene: 1832 Human

Omim: 125647 Human

SwissProt: P15924 Human

Unigene: 519873 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications
   
   

桥粒是在相邻细胞之间形成紧密连接的细胞间连接。桥粒蛋白是功能性桥粒的必需成分，将中间丝附着在桥粒斑块上。它涉及到组织中的桥粒钙粘蛋白-血小板蛋白复合物到离散的质膜结构域。桥粒蛋白的N-端对于桥粒的定位以及与亲斑蛋白1和斑珠蛋白的相互作用是必不可少的。桥莫司汀的C末端与中间丝结合。DeMPLAKIN的中心区域包括介导同质二聚化的卷曲螺旋棒结构域。有两种同种形式的结缔组织蛋白酶-结缔组织蛋白酶I，它是所有结缔体的必需成分，以及结缔组织蛋白酶II，它主要表达在分层来源的组织和细胞中。编码桥粒蛋白基因的突变导致许多心肌病和角化皮瘤，以及自身免疫性疾病副肿瘤性天疱疮。