产品展示
ED-1外胚层发育不良抗体
点击次数:233发布时间:2012/12/5 14:47:29
更新日期:2024/9/5 14:43:01
所 在 地:中国大陆
产品型号:BY-1149R
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详细内容
货号:BY-1149R
英文名称:Anti-ED-1
中文名称:外胚层发育不良抗体
其他名称:名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 protein homolog; EDA1; EDA1 GENE; EDA2; HED; Ta; Tabby; Tabby protein; XHED; XLHED.
抗体来源:Rabbit
克隆类型:polyclonal
蛋白分子量:predicted molecular weight: 42kDa
纯化方法:affinity purified by Protein A
交叉反应:hu, rat, mo
产品介绍: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.Subcellular Location : Secreted and Cell membrane.Tissue Specificity : Not abundant; expressed in specific cell types of ectodermal (but not mesodermal) origin of keratinocytes, hair follicles, sweat glands. Also in adult heart, liver, muscle, pancreas, prostate, fetal liver, uterus, small intestine and umbilical chord.Post-translational modifications : Processing by furin produces a secreted form.DISEASE : Defects in EDA are the cause of ectodermal dysplasia type 1 (ED1) [MIM:305100]; also known as Christ-Siemens-Touraine syndrome or X-linked hypohidrotic ectodermal dysplasia (XLHED). Ectodermal dysplasia defines a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. ED1 is a disease characterized by sparse hair (atrichosis or hypotrichosis), abnormal or missing teeth and the inability to sweat due to the absence of sweat glands. ED1 is the most common form of over 150 clinically distinct ectodermal dysplasias.Similarity : Belongs to the tumor necrosis factor family.Contains 1 collagen-like domain. Ectodermal dysplasia 1属于TNF家族成员,为常染色体隐性遗传病。产品图片Antigen: bs-1149P, 0.2ug/100ul Primary: Antiserum, 1:500, 1:1000, 1:2000, 1:4000, 1:8000, 1:16000, 1:32000; Secondary: HRP conjugated Goat-Anti-Rabbit IgG(bs-0295G-HRP) at 1: 5000; TMB(C-0024) staining; Read the data in MicroplateReader by 450nm.