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Rabbit Anti-EVER2/BF350 Conjugated antibody (bs-13116R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-13116R-BF350
英文名稱1 Rabbit Anti-EVER2/BF350 Conjugated antibody
中文名稱 BF350標記的跨膜通道蛋白8抗體
別    名 epidermodysplasia verruciformis 2; Epidermodysplasia verruciformis protein 2; EV2; EVIN2; FLJ40668; FLJ43684; MGC102701; MGC40121; TMC8; Transmembrane channel like protein 8; TMC8_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  跨膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, 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 82kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human EVER2/TMC8
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:
Transmembrane channel-like protein 8 (TMC8), also known as Epidermodysplasia verruciformis protein 2 (EVER2), is a 726 amino acid member of the TMC family of proteins. Localized to the endoplasmic reticulum membrane, TMC8 is thought to form a transmembrane channel-like protein with eight predicted transmembrane domains and three leucine zipper motifs. Mutations in the genes encoding TMC8 and TMC6, another member of the TMC family, have been shown to cause epidermodysplasia verruciformis (EV), an autosomal recessive dermatosis characterized by abnormal susceptibility to human papillomaviruses (HPVs) and a high rate of progression to squamous cell carcinoma on sun-exposed skin. Infection by HPVs lead to persistent wart-like or macular lesions. TMC8 is expressed in placenta, prostate and testis, and three named isoforms exist as a result of alternative splicing events.

Function:
Epidermodysplasia verruciformis (EV) is an autosomal recessive genodermatosis associated with a high risk of skin cancers resulting from a high susceptibility to infection by specific human papillomaviruses. Mutations in two homologous genes EVER1 and EVER2 cause the majority of EV cases. These two proteins form a complex and interact with the zinc transporter ZnT-1 in the endoplasmic reticulum. Cells lacking EVER2 accumulated higher levels of zinc in the nucleolus and nucleus compare to those cells with and intact EVER2 gene, indicating that one role of EVER2 is to regulate the intracellular distribution of zinc. At least two isoforms of EVER2 are known to exist.

Subcellular Location:
Endoplasmic reticulum membrane; Multi pass membrane protein.

Tissue Specificity:
Expressed in placenta, prostate and testis.

DISEASE:
Defects in TMC8 are a cause of epidermodysplasia verruciformis (EV) [MIM:226400]. It is a rare autosomal recessive genodermatosis associated with a high risk of skin carcinoma that results from an abnormal susceptibility to infection by specific human papillomaviruses. Infection leads to persistent wart-like or macular lesions.

Similarity:
Belongs to the TMC family.

Database links:

Entrez Gene: 147138 Human

Entrez Gene: 217356 Mouse

Omim: 605829 Human

SwissProt: Q8IU68 Human

SwissProt: Q7TN58 Mouse

Unigene: 592102 Human



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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