| 產(chǎn)品編號 | bs-17493R-BF350 |
| 英文名稱 | Rabbit Anti-SIL1/BF350 Conjugated antibody |
| 中文名稱 | BF350標(biāo)記的內(nèi)質(zhì)網(wǎng)分子伴侶SIL1抗體 |
| 別 名 | BAP; BiP associated protein; BiP-associated protein; Endoplasmic reticulum chaperone SIL 1; Endoplasmic reticulum chaperone SIL1; MSS; Nucleotide exchange factor SIL 1; Nucleotide exchange factor SIL1; SIL 1; sil1; SIL1 homolog; SIL1 homolog endoplasmic reticulum chaperone (S. cerevisiae); SIL1 homolog endoplasmic reticulum chaperone; SIL1_HUMAN; UGL 5; ULG5. |
| 規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 細胞生物 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) 細胞凋亡 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse, (predicted: Human, Rat, Dog, Pig, Horse, ) |
| 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 | 49kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human SIL1 |
| 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(chǎn)品介紹 |
background: SIKE interacts with IKK-epsilon (IKBKE; MIM 605048) and TBK1 (MIM 604834) and acts as a suppressor of TLR3 (MIM 603029) and virus-triggered interferon activation pathways (Huang et al., 2005 [PubMed 16281057]).[supplied by OMIM, Mar 2008] Function: Required for protein translocation and folding in the endoplasmic reticulum (ER). Functions as a nucleotide exchange factor for the ER lumenal chaperone HSPA5. Subcellular Location: Endoplasmic reticulum lumen. Tissue Specificity: Highly expressed in tissues which produce large amounts of secreted proteins such as kidney, liver and placenta. Also expressed in colon, heart, lung, ovary, pancreas, peripheral leukocyte, prostate, spleen and thymus. Expressed at low levels throughout the brain. Post-translational modifications: N-glycosylated. DISEASE: Defects in SIL1 are a cause of Marinesco-Sjoegren syndrome (MSS) [MIM:248800]. MSS is an autosomal recessive multisystem disorder which is characterized by cerebellar ataxia due to cerebellar atrophy, with Purkinje and granule cell loss and myopathy featuring marked muscle replacement with fat and connective tissue. Other cardinal features include bilateral cataracts, hypergonadotrophic hypogonadism and mild to severe mental retardation. Skeletal abnormalities, short stature, dysarthria, strabismus and nystagmus are also frequent findings. Mutational inactivation of this protein may result in ER stress-induced cell death signaling or malfunctioning chaperone machineries that mishandle client proteins which are critical for the organs targeted in MSS. Similarity: Belongs to the SIL1 family. Database links: Entrez Gene: 64374 Human Entrez Gene: 81500 Mouse Omim: 608005 Human SwissProt: Q9H173 Human SwissProt: Q9EPK6 Mouse Unigene: 483521 Human Unigene: 291482 Mouse Unigene: 103851 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
