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UBA5 Antibody

  • 货号：
    CSB-PA004356
  • 规格：
    ￥880
  • 图片：
    • Western Blot analysis of 293 cells using Uba5 Polyclonal Antibody
  • 其他：

产品详情

  • Uniprot No.：
    Q9GZZ9
  • 基因名：
    UBA5
  • 别名：
    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
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Synthesized peptide derived from the C-terminal region of Human Uba5.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, IHC, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:100-1:300
    ELISA 1:5000
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Epileptic encephalopathy, early infantile, 44 (EIEE44); Spinocerebellar ataxia, autosomal recessive, 24 (SCAR24)
  • 亚细胞定位：
    Cytoplasm. Nucleus. Endoplasmic reticulum membrane. Golgi apparatus.
  • 蛋白家族：
    Ubiquitin-activating E1 family, UBA5 subfamily
  • 组织特异性：
    Widely expressed.
  • 数据库链接：

    HGNC: 23230

    OMIM: 610552

    KEGG: hsa:79876

    STRING: 9606.ENSP00000348565

    UniGene: Hs.170737