产品名称：Anti-EDA/FITC抗体

Anti-EDA/FITC抗体

产品编号YB-1149R-FITC

英文名称Anti-EDA/FITC

中文名称FITC标记的外胚层发育**蛋白抗体

别    名Ectodysplasin-A; Ectodermal dysplasia 1, anhidrotic; Ectodermal dysplasia protein; Ectodermal dysplasia, anhidrotic (hypohydrotic); Ectodysplasin A; Ectodysplasin A, membrane form; Ectodysplasin A, secreted form; ECTODYSPLASIN A1 ISOFORM; ECTODYSPLASIN A2 ISOFORM; ECTODYSPLASIN; ED1 A1; ED1 A2; ED1; ED1 GENE; Eda A1; Eda A2; EDA protein; EDA_HUMAN; ODT1; Oligodontia 1; secreted form; STHAGX1; Ta; Tabby; Tabby protein; X linked anhidroitic ectodermal dysplasia protein; XHED; XLHED; EDA protein homolog; EDA1; EDA1 GENE; EDA2; HED; Ta; Tabby; Tabby protein; XHED; XLHED.  

Anti-EDA/FITC抗体

说 明 书100ul  

研究领域细胞生物  **学  信号转导  

抗体来源Rabbit

克隆类型Polyclonal

交叉反应 Human, Mouse, Rat, Pig, Cow, Horse,

产品应用ICC=1:50-200 IF=1:50-200  

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

分 子 量24/41kDa

细胞定位细胞膜

Anti-EDA/FITC抗体性    状Lyophilized or Liquid

浓    度1mg/ml

免 疫 原KLH conjugated synthetic peptide derived from human Ectodysplasin-A, secreted form

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.

Anti-EDA/FITC抗体

产品介绍background:

The protein encoded by the EDA gene is a type II membrane protein that can be cleaved by furin to produce a secreted form. The encoded protein, which belongs to the tumor necrosis factor family, acts as a homotrimer and may be involved in cell-cell signaling during the development of ectodermal organs. Defects in the EDA gene are a cause of ectodermal dysplasia, anhidrotic, (this is also known as X-linked hypohidrotic ectodermal dysplasia). Several transcript variants encoding many different isoforms have been found for this gene. AltName: ectodermal dysplasia protein; Ectodysplasin-A; EDA protein homolog; Tabby protein; Ectodysplasin-A, membrane form; secreted form EDA-A5.

Function:

Seems to be involved in epithelial-mesenchymal signaling during morphogenesis of ectodermal organs. Isoform 1 binds only to the receptor EDAR, while isoform 3 binds exclusively to the receptor XEDAR.

Subunit:

Homotrimer. The homotrimers may then dimerize and form higher-order oligomers.

Subcellular Location:

Cell membrane; Single-pass type II membrane protein.

Ectodysplasin-A, secreted form: Secreted.

Tissue Specificity:

Not abundant; expressed in specific cell types oAnti-EDA/FITC抗体f ectodermal (but not mesodermal) origin of keratinocytes, hair follicles, sweat glands. Also in ***** heart, liver, muscle, pancreas, prostate, fetal liver, uterus, small intestine and umbilical chord.

Post-translational modifications:

N-glycosylated.

Processing by furin produces a secreted form.

DISEASE:

Ectodermal dysplasia 1, hypohidrotic, X-linked (XHED) [MIM:305100]: A form of ectodermal dysplasia, a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. Characterized by sparse hair (atrichosis or hypotrichosis), abnormal or missing teeth and the inability to sweat due to the absence of sweat glands. It is the most common form of over 150 clinically distinct ectodermal dysplasias. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tooth agenesis selective X-linked 1 (STHAGX1) [MIM:313500]: A form of selective tooth agenesis, a common anomaly characterized by the congenital absence of one or more teeth. Selective tooth agenesis without associated systemic disorders has sometimes been divided into 2 types: oligodontia, defined as agenesis of 6 or more permanent teeth, and hypodontia, defined as agenesis of less than 6 teeth. The number in both cases does not include absence of third molars (wisdom teeth). Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:

Belongs to the tumor necrosis factor family.

Contains 1 collagen-like domain.

Database links:

Entrez Gene: 1896 Human

Entrez Gene: 13607 Mouse

Entrez Gene: 302424 Rat

Omim: 300451 Human

SwissProt: Q92838 Human

SwissProt: O54693 Mouse

Unigene: 105407 Human

Unigene: 328086 Mouse

Unigene: 211298 Rat

Important Note:

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

Ectodermal dysplasia 1属于TNF家族成员， 为常染色体隐性遗传病。