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UBR1 Antibody, FITC conjugated

  • 中文名稱：
    UBR1兔多克隆抗體, FITC偶聯(lián)
  • 貨號：
    CSB-PA812883LC01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) UBR1 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    UBR1
  • 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
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human E3 ubiquitin-protein ligase UBR1 protein (722-862AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    FITC
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 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
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 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.

產(chǎn)品評價(jià)

靶點(diǎn)詳情

  • 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.
  • 基因功能參考文獻(xiàn)：
    1. UBR1 mutations of single or multi-exon deletions or duplications account for a substantial proportion of Johanson-Blizzard syndrome. PMID: 29178640
    2. The frequency of any non-synonymous or synonymous variants was not different between the patients with chronic pancreatitis and controls PMID: 27397733
    3. Reduced UBR1 expression affects MGMT turnover and DNA repair in the smokers lungs. PMID: 26183928
    4. For all previously reported and novel UBR1 mutations together with their clinical data, a mutation database has been established at LOVD. PMID: 24599544
    5. Testing the fetus and the affected sibling with recurrent Johanson-Blizzard syndrome revealed a homozygous truncating mutation in UBR1. PMID: 21711208
    6. 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
    7. 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
    8. Ubc2/Rad6 ser(120) regulates ubiquitin-dependent N-end rule targeting by E3{alpha}/Ubr1 PMID: 21041297
    9. 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
    10. Sustained ERK5 activity and the E3 ligase UBR1 regulate the stability and subcellular localization of c-Fos. PMID: 17018293
    11. Thus we propose that autoISGylation of EFP negatively regulates its ISG15 E3 ligase activity for 14-3-3sigma. PMID: 17222803
    12. These results suggest that Rabring7 is involved in the endocytic trafficking of EGFR through its E3 ligase activity. PMID: 17462600
    13. Thiazolidinediones modulate the expression of beta catenin and other cell cycle proteins by targeting UBR1 independently of PPARG. PMID: 17569795
    14. E3 ubiquitin ligase is an essential downstream component of the RAS signal transduction pathway. PMID: 18089810
    15. 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
    16. 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)
  • 亞細(xì)胞定位：
    Cytoplasm, cytosol.
  • 蛋白家族：
    UBR1 family
  • 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).
  • 數(shù)據(jù)庫鏈接：

    HGNC: 16808

    OMIM: 243800

    KEGG: hsa:197131

    STRING: 9606.ENSP00000290650

    UniGene: Hs.591121