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Rabbit Anti-EDARADD/BF350 Conjugated antibody (bs-14498R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-14498R-BF350
英文名稱1 Rabbit Anti-EDARADD/BF350 Conjugated antibody
中文名稱 BF350標記的少汗型外胚層發(fā)育不良相關蛋白EDAD抗體
別    名 Cr; Crinkled homolog; ectodysplasia A receptor associated death domain; Ectodysplasin-A receptor-associated adapter protein; ED3; EDA3; EDAD_HUMAN; EDAR associated death domain; EDAR-associated death domain protein; EDARADD; Protein crinkled homolog.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 發(fā)育生物學  信號轉導  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep, )
產品應用 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 25kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human EDARADD
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產品介紹 background:
This gene was identified by its association with ectodermal dysplasia, a genetic disorder characterized by defective development of hair, teeth, and eccrine sweat glands. The protein encoded by this gene is a death domain-containing protein, and is found to interact with EDAR, a death domain receptor known to be required for the development of hair, teeth and other ectodermal derivatives. This protein and EDAR are coexpressed in epithelial cells during the formation of hair follicles and teeth. Through its interaction with EDAR, this protein acts as an adaptor, and links the receptor to downstream signaling pathways. Two alternatively spliced transcript variants of this gene encoding distinct isoforms have been reported. [provided by RefSeq, Jul 2008]

Function:
Adapter protein that interacts with EDAR DEATH domain and couples the receptor to EDA signaling pathway during morphogenesis of ectodermal organs. Mediates the activation of NF-kappa-B.

Subcellular Location:
Cytoplasm.

Tissue Specificity:
Detected in adult pancreas, placenta and fetal skin, and at lower levels in lung, thymus, prostate and testis.

DISEASE:
Defects in EDARADD are a cause of ectodermal dysplasia anhidrotic (EDA) [MIM:224900]; also known ectodermal dysplasia hypohidrotic autosomal recessive (HED). Ectodermal dysplasia defines a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. EDA is characterized by sparse hair (atrichosis or hypotrichosis), abnormal or missing teeth and the inability to sweat due to the absence of sweat glands.

Similarity:
Contains 1 death domain.

Database links:

Entrez Gene: 128178 Human

Entrez Gene: 171211 Mouse

Entrez Gene: 498769 Rat

Omim: 606603 Human

SwissProt: Q8WWZ3 Human

SwissProt: Q8VHX2 Mouse

Unigene: 352224 Human

Unigene: 159671 Mouse



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