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UBA5 Antibody

  • 中文名稱(chēng):
    UBA5兔多克隆抗體
  • 貨號(hào):
    CSB-PA875638LA01HU
  • 規(guī)格:
    ¥440
  • 圖片:
    • Western blot
      All lanes: UBA5 antibody at 6μg/ml
      Lane 1: Jurkat whole cell lysate
      Lane 2: Hela whole cell lysate
      Lane 3: HepG2 whole cell lysate
      Lane 4: Caco-2 whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 45, 39 kDa
      Observed band size: 45 kDa
  • 其他:

產(chǎn)品詳情

  • 產(chǎn)品名稱(chēng):
    Rabbit anti-Homo sapiens (Human) UBA5 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    UBA5
  • 別名:
    5730525G14Rik antibody; AW240750 antibody; FLJ23251 antibody; FLJ23251UBA5 antibody; MGC105565 antibody; ThiFP1 antibody; UBA5 antibody; UBA5 ubiquitin activating enzyme E1 homolog antibody; UBA5_HUMAN antibody; UBE1DC antibody; UBE1DC1 antibody; Ubiquitin activating enzyme 5 antibody; Ubiquitin activating enzyme E1 domain containing 1 antibody; Ubiquitin activating enzyme E1 domain containing protein 1 antibody; Ubiquitin like modifier activating enzyme 5 antibody; Ubiquitin-activating enzyme 5 antibody; Ubiquitin-activating enzyme E1 domain-containing protein 1 antibody; Ubiquitin-like modifier-activating enzyme 5 antibody; UFM1 activating enzyme antibody; UFM1-activating enzyme antibody
  • 宿主:
    Rabbit
  • 反應(yīng)種屬:
    Human
  • 免疫原:
    Recombinant Human Ubiquitin-like modifier-activating enzyme 5 protein (291-404AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標(biāo)記方式:
    Non-conjugated

    本頁(yè)面中的產(chǎn)品,UBA5 Antibody (CSB-PA875638LA01HU),的標(biāo)記方式是Non-conjugated。對(duì)于UBA5 Antibody,我們還提供其他標(biāo)記。見(jiàn)下表:

    可提供標(biāo)記
    標(biāo)記方式 貨號(hào) 產(chǎn)品名稱(chēng) 應(yīng)用
    HRP CSB-PA875638LB01HU UBA5 Antibody, HRP conjugated ELISA
    FITC CSB-PA875638LC01HU UBA5 Antibody, FITC conjugated
    Biotin CSB-PA875638LD01HU UBA5 Antibody, Biotin conjugated ELISA
  • 克隆類(lèi)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
  • 應(yīng)用范圍:
    ELISA, WB
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:2000
  • Protocols:
  • 儲(chǔ)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.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能:
    E1-like enzyme which specifically catalyzes the first step in ufmylation. Activates UFM1 by first adenylating its C-terminal glycine residue with ATP, and thereafter linking this residue to the side chain of a cysteine residue in E1, yielding a UFM1-E1 thioester and free AMP. Activates UFM1 via a trans-binding mechanism, in which UFM1 interacts with distinct sites in both subunits of the UBA5 homodimer. Trans-binding also promotes stabilization of the UBA5 homodimer, and enhances ATP-binding. Transfer of UFM1 from UBA5 to the E2-like enzyme UFC1 also takes place using a trans mechanism. Ufmylation is involved in reticulophagy (also called ER-phagy) induced in response to endoplasmic reticulum stress. Ufmylation is essential for erythroid differentiation of both megakaryocytes and erythrocytes.
  • 基因功能參考文獻(xiàn):
    1. UFM1 His 70 resembles UBA5 His336 and enters a negatively charged pocked on the other UFM1 molecule. PMID: 28360427
    2. These findings explicitly elucidate the role of UBA5 dimerization in UFM1 activation. PMID: 27653677
    3. Heterozygous mutations in the UBA5 gene in two sisters with early-onset epileptic encephalopathy . PMID: 28965491
    4. Biallelic Variants in UBA5 Link Dysfunctional UFM1 Ubiquitin-like Modifier Pathway to Severe Infantile-Onset Encephalopathy PMID: 27545674
    5. clinical, biochemical, and experimental findings support our finding of UBA5 mutations as a pathophysiological cause for early-onset encephalopathies due to abnormal protein ufmylation PMID: 27545681
    6. study provides important structural and functional insights into the interaction between UBA5 and Ub-like modifiers, improving the understanding of the biology of the ufmylation pathway. PMID: 26929408
    7. The study of UBA5-R246X revealed a dramatically decreased half-life and loss of UFM1 activation due to the absence of the catalytic cysteine Cys250. PMID: 26872069
    8. Uba5 residues 364-404 were demonstrated to be necessary for the transthiolation of Ufm1 to Ufc1, and Uba5 381-404 was identified to be the minimal region for Ufc1 recognition PMID: 25084390
    9. Crystallization of Uba5 residues 57-363 was performed at 277 K using PEG 3350 as the precipitant, and crystals optimized by microseeding diffracted to 2.95 A resolution, with unit-cell parameters a=b=97.66, c=144.83 A, alpha=beta=90, gamma=120 degrees . PMID: 24915089
    10. binding of ATP to Uba5 approximately Ufm1 thioester was required for efficient transfer of Ufm1 from Uba5 to Ufc1 via transthiolation. PMID: 24966333
    11. Studies reveal structural features of UBA5 that further understanding of the enzyme reaction mechanism and provide insight into the evolution of ubiquitin activation. PMID: 20368332
    12. UBE1DC1 greatly activated SUMO2 in the nucleus or transferred activated-SUMO2 to nucleus after it conjugated SUMO2 in the cytoplasm. PMID: 18442052

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  • 相關(guān)疾病:
    Epileptic encephalopathy, early infantile, 44 (EIEE44); Spinocerebellar ataxia, autosomal recessive, 24 (SCAR24)
  • 亞細(xì)胞定位:
    Cytoplasm. Nucleus. Endoplasmic reticulum membrane. Golgi apparatus.
  • 蛋白家族:
    Ubiquitin-activating E1 family, UBA5 subfamily
  • 組織特異性:
    Widely expressed.
  • 數(shù)據(jù)庫(kù)鏈接:

    HGNC: 23230

    OMIM: 610552

    KEGG: hsa:79876

    STRING: 9606.ENSP00000348565

    UniGene: Hs.170737



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