SIL1 Antibody
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中文名稱：SIL1兔多克隆抗體
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貨號(hào)：CSB-PA875645LA01HU
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規(guī)格：￥440
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圖片：
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Western blot
All lanes: SIL1 antibody at 2μg/ml + Mouse kidney tissue
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 52 kDa
Observed band size: 52 kDa
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其他：
產(chǎn)品詳情
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產(chǎn)品名稱：Rabbit anti-Homo sapiens (Human) SIL1 Polyclonal antibody
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Uniprot No.：
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基因名：
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別名：BAP antibody; BiP associated protein antibody; BiP-associated protein antibody; Endoplasmic reticulum chaperone SIL 1 antibody; Endoplasmic reticulum chaperone SIL1 antibody; MSS antibody; Nucleotide exchange factor SIL 1 antibody; Nucleotide exchange factor SIL1 antibody; SIL 1 antibody; sil1 antibody; SIL1 homolog antibody; SIL1 homolog endoplasmic reticulum chaperone (S. cerevisiae) antibody; SIL1 homolog endoplasmic reticulum chaperone antibody; SIL1_HUMAN antibody; UGL 5 antibody; ULG5 antibody
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宿主：Rabbit
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反應(yīng)種屬：Human, Mouse
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免疫原：Recombinant Human Nucleotide exchange factor SIL1 protein (32-174AA)
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免疫原種屬：Homo sapiens (Human)
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標(biāo)記方式：Non-conjugated
本頁(yè)面中的產(chǎn)品，SIL1 Antibody (CSB-PA875645LA01HU)，的標(biāo)記方式是Non-conjugated。對(duì)于SIL1 Antibody，我們還提供其他標(biāo)記。見(jiàn)下表：
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：>95%, Protein G purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 -
產(chǎn)品提供形式：Liquid
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應(yīng)用范圍：ELISA, WB
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推薦稀釋比：
Application Recommended Dilution WB 1:200-1:1000 -
Protocols：
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儲(chǔ)存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情
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功能：Required for protein translocation and folding in the endoplasmic reticulum (ER). Functions as a nucleotide exchange factor for the ER lumenal chaperone HSPA5.
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基因功能參考文獻(xiàn)：
- SIL1-depleted HEK293 cells are an appropriate model to identify proteins modulated by SIL1 expression level. PMID: 26468156
- In a child with Marinesco-Sjogren syndrome it was found that a mutation in SIL1 affected the 5' UTR, translation initiation site and the endoplasmic reticulum-targeting signal sequence. PMID: 27544240
- this case study is the first report on Chinese Marinesco-Sjogren syndrome (MSS) patients, MSS complicated by Dandy-Walker syndrome (DWS), and a nonstop mutation in SIL1; our findings imply the pathogenetic association between DWS and MSS PMID: 27106665
- Two NEFs, Grp170 and Sil1, trigger toxin release from BiP to enable successful retrotranslocation and clarify the fate of the toxin after it disengages from BiP. PMID: 25877869
- This study demonistrated that SIL1 mutation in patient with ataxia telangiectasia PMID: 24631270
- The mutations prevent SIL1 from interacting with and regulating HSPA5, leading to abnormal neuronal morphology and migration. PMID: 24473200
- The study confirms the previous findings of mutations in SIL1 being the major cause of Marinesco-Sjogren syndrome. PMID: 24176978
- The clinical features and two novel SIL1 mutations of four Dutch patients with Marinesco-Sjogren syndrome are described PMID: 23062754
- the very C-terminal residues of SIL1 play a role in its structural integrity rather than its localization. PMID: 22219183
- The patients described here manifested the cardinal features of Marinesco-Sjogren syndrome, but did not exhibit any mutation in the exons and flanking introns of the SIL1 gene. PMID: 22115007
- Some reported cases of Marinesco-Sjogren syndrome without base alterations in the SIL1 gene are caused by deletions rather than locus heterogeneity. PMID: 20111056
- Interactions between Kar2p and its nucleotide exchange factors Sil1p and Lhs1p are mechanistically distinct PMID: 20430899
- BAP serves as a nucleotide exchange factor for BiP PMID: 12356756
- Nine distinct mutations that would disrupt the SIL1 protein in individuals with Marinesco-Sjogren syndrome were identified. PMID: 16282977
- Four Marinesco-Sjogren syndrome-associated loss-of-function mutations in SIL1 leading to disturbed SIL1-HSPA5 interaction and protein folding were identified. PMID: 16282978
- A novel mutation in BAP/SIL1 gene causes Marinesco-Sjogren syndrome in an extended pedigree. PMID: 17026626
- SIL1 mutation is associated with Marinesco-Sjogren syndrome PMID: 18285827
- We sequenced the entire SIL1-coding region in 3 unrelated Japanese patients with classical MSS and identified a novel homozygous frameshift insertion mutation, 936_937insG, in exon 9 in all 3 patients. PMID: 18395226
- data report two novel SIL1 missense mutations in two consanguineous Pakistani families affected with Marinesco-Sjogren syndrome PMID: 19471582
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相關(guān)疾病：Marinesco-Sjoegren syndrome (MSS)
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亞細(xì)胞定位：Endoplasmic reticulum lumen.
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蛋白家族：SIL1 family
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組織特異性：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 t
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