| 產(chǎn)品編號(hào) | bs-14406R-BF350 |
| 英文名稱1 | Rabbit Anti-DOLK/BF350 Conjugated antibody |
| 中文名稱 | BF350標(biāo)記的TMEM15/跨膜蛋白15抗體 |
| 別 名 | CDG1M; DK; DK1; Dolichol kinase; KIAA1094; DOLK_HUMAN; SEC59; SEC59, YEAST, HOMOLOG OF; TMEM15; Transmembrane protein 15. |
| 規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 細(xì)胞生物 跨膜蛋白 細(xì)胞膜蛋白 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse, (predicted: Human, Rat, Chicken, Pig, Cow, Horse, Sheep, ) |
| 產(chǎn)品應(yīng)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 | 59kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human DOLK |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 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(chǎn)品介紹 |
background: The protein encoded by this gene catalyzes the CTP-mediated phosphorylation of dolichol, and is involved in the synthesis of Dol-P-Man, which is an essential glycosyl carrier lipid for C- and O-mannosylation, N- and O-linked glycosylation of proteins, and for the biosynthesis of glycosyl phosphatidylinositol anchors in endoplasmic reticulum. Mutations in this gene are associated with dolichol kinase deficiency.[provided by RefSeq, Apr 2010] Function: DOLK belongs to the polyprenol kinase family. Defects in DOLK are the cause of congenital disorder of glycosylation type 1M (CDG1M), also known as dolichol kinase deficiency. Subcellular Location: Endoplasmic reticulum integral membrane protein Tissue Specificity: Ubiquitous. DISEASE: Congenital disorder of glycosylation 1M (CDG1M) [MIM:610768]: A multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. CDG1M is a very severe disease with death occurring in early life. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the polyprenol kinase family. Database links: Entrez Gene: 22845 Human Omim: 610746 Human SwissProt: Q9UPQ8 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
