UBR1 Antibody, FITC conjugated
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中文名稱:UBR1兔多克隆抗體, FITC偶聯(lián)
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貨號:CSB-PA812883LC01HU
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規(guī)格:¥880
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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) UBR1 Polyclonal antibody
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Uniprot No.:
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基因名:UBR1
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別名:E3 ubiquitin-protein ligase UBR1 antibody; JBS antibody; N-recognin-1 antibody; ubiquitin protein ligase E3 component n-recognin 1 antibody; Ubiquitin-protein ligase E3-alpha-1 antibody; Ubiquitin-protein ligase E3-alpha-I antibody; UBR1 antibody; UBR1_HUMAN antibody
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宿主:Rabbit
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反應(yīng)種屬:Human
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免疫原:Recombinant Human E3 ubiquitin-protein ligase UBR1 protein (722-862AA)
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免疫原種屬:Homo sapiens (Human)
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標(biāo)記方式:FITC
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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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儲存條件: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.
相關(guān)產(chǎn)品
靶點(diǎn)詳情
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功能:E3 ubiquitin-protein ligase which is a component of the N-end rule pathway. Recognizes and binds to proteins bearing specific N-terminal residues that are destabilizing according to the N-end rule, leading to their ubiquitination and subsequent degradation. May be involved in pancreatic homeostasis. Binds leucine and is a negative regulator of the leucine-mTOR signaling pathway, thereby controlling cell growth.
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基因功能參考文獻(xiàn):
- UBR1 mutations of single or multi-exon deletions or duplications account for a substantial proportion of Johanson-Blizzard syndrome. PMID: 29178640
- The frequency of any non-synonymous or synonymous variants was not different between the patients with chronic pancreatitis and controls PMID: 27397733
- Reduced UBR1 expression affects MGMT turnover and DNA repair in the smokers lungs. PMID: 26183928
- For all previously reported and novel UBR1 mutations together with their clinical data, a mutation database has been established at LOVD. PMID: 24599544
- Testing the fetus and the affected sibling with recurrent Johanson-Blizzard syndrome revealed a homozygous truncating mutation in UBR1. PMID: 21711208
- Results confirmed the relevance of specific missense UBR1 alleles to JBS, and suggested that a residual activity of a missense allele is causally associated with milder variants of JBS. PMID: 21931868
- Molecular studies revealed a novel homozygous nonsense mutation in exon 38 of the UBR1 gene, which confirmed the diagnosis of Johanson-Blizzard syndrome. PMID: 20556423
- Ubc2/Rad6 ser(120) regulates ubiquitin-dependent N-end rule targeting by E3{alpha}/Ubr1 PMID: 21041297
- Deficiency of UBR1 perturbs the pancreas' acinar cells and other organs, presumably owing to metabolic stabilization of specific substrates of the N-end rule pathway. PMID: 16311597
- Sustained ERK5 activity and the E3 ligase UBR1 regulate the stability and subcellular localization of c-Fos. PMID: 17018293
- Thus we propose that autoISGylation of EFP negatively regulates its ISG15 E3 ligase activity for 14-3-3sigma. PMID: 17222803
- These results suggest that Rabring7 is involved in the endocytic trafficking of EGFR through its E3 ligase activity. PMID: 17462600
- Thiazolidinediones modulate the expression of beta catenin and other cell cycle proteins by targeting UBR1 independently of PPARG. PMID: 17569795
- E3 ubiquitin ligase is an essential downstream component of the RAS signal transduction pathway. PMID: 18089810
- study reports on two apparently unrelated girls with Johanson-Blizzard syndrome, in both children caused by a homozygous IVS26+5G>A mutation in the UBR1 gene PMID: 19006206
- Case Report: Johanson-Blizzard syndrome with mild phenotypic features confirmed by UBR1 gene testing. PMID: 19058315
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相關(guān)疾病:Johanson-Blizzard syndrome (JBS)
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亞細(xì)胞定位:Cytoplasm, cytosol.
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蛋白家族:UBR1 family
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組織特異性:Broadly expressed, with highest levels in skeletal muscle, kidney and pancreas. Present in acinar cells of the pancreas (at protein level).
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