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ELP1 Antibody, FITC conjugated

  • 中文名稱:
    ELP1兔多克隆抗體, FITC偶聯(lián)
  • 貨號:
    CSB-PA011571LC01HU
  • 規(guī)格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) ELP1 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    ELP1
  • 別名:
    DKFZp781H1425 antibody; DYS antibody; Dysautonomia (Riley Day syndrome hereditary sensory autonomic neuropathy type III) antibody; Elongator complex protein 1 antibody; ELP 1 antibody; ELP1 antibody; ELP1_HUMAN antibody; FD antibody; FLJ12497 antibody; IKAP antibody; IkappaB kinase complex associated protein antibody; IkappaB kinase complex-associated protein antibody; ikbkap antibody; IKI 3 antibody; IKI3 antibody; IKK complex associated protein antibody; IKK complex-associated protein antibody; Inhibitor of kappa light polypeptide gene enhancer in B cells kinase complex associated protein antibody; OTTHUMP00000063889 antibody; p150 antibody; TOT 1 antibody; TOT1 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Elongator complex protein 1 protein (1107-1258AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    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.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能:
    Component of the RNA polymerase II elongator complex, a multiprotein complex associated with the RNA polymerase II (Pol II) holoenzyme, and which is involved in transcriptional elongation. The elongator complex catalyzes formation of carboxymethyluridine in the wobble base at position 34 in tRNAs. Involved in neurogenesis. Regulates the migration and branching of projection neurons in the developing cerebral cortex, through a process depending on alpha-tubulin acetylation. May act as a scaffold protein that may assemble active IKK-MAP3K14 complexes (IKKA, IKKB and MAP3K14/NIK).
  • 基因功能參考文獻:
    1. overexpression of miR-203a-3p leads to a decrease of NOVA1, counter-balanced by an increase of IKAP, supporting a potential interaction between NOVA1 and IKAP. PMID: 27483351
    2. IKAP might be a vesicular like protein that might be involved in neuronal transport in hESC derived PNS neurons PMID: 26437462
    3. The formation of the Elp1 dimer contributes to its stability in vitro and in vivo and is required for the assembly of human Elongator complexes. PMID: 26261306
    4. IKBKAP mRNA levels decreased during a familial dysautonomia crisis and returned to baseline after recovery. The cause-and-effect relationship is unclear. PMID: 24268683
    5. Phosphatidylserine increases IKBKAP levels in a humanized knock-in IKBKAP mouse model for Familial dysautonomia. PMID: 23515154
    6. Digoxin-mediated repression of SRSF3 expression plays a role in the digoxin-mediated inclusion of exon 20 in the IKBKAP transcript generated from the familial dysautonomia mutant allele. PMID: 23711097
    7. Combined treatment with epigallocatechin gallate and genistein synergistically upregulates wild-type IKBKAP-encoded RNA and protein levels in familial dysautonomia-derived cells. PMID: 22495984
    8. IKAP plays pleiotropic roles in both the peripheral and central nervous systems PMID: 22384137
    9. IKAP/hELP1 deficiency has an effect on gene expression in differentiating neuroblastoma cells, and possibly on familial dysautonomia PMID: 21559466
    10. IKK complex-associated protein deficiency upregulates the microtubule destabilizing protein SCG10 and, in parallel, disorganizes the cytoskeleton PMID: 21273291
    11. Phosphatidylserine increases IKBKAP levels in familial dysautonomia cells PMID: 21209961
    12. IKAP regulates contactin levels for appropriate cell-cell adhesion that could modulate neuronal growth of neurons during development PMID: 20671422
    13. IKAP is critical for the development of afferent baroreflex pathways and has therapeutic implications in the management of these patients. PMID: 21098405
    14. IKBKAP is a candidate gene for Hirschsprung's disease and was mapped to chromosome 9q31 locus. PMID: 20361209
    15. novel role for the I kappa B kinase complex-associated protein (IKAP) in the regulation of activation of the mammalian stress response via the c-Jun N-terminal kinase (JNK)-signaling pathway PMID: 12058026
    16. Genetics of familial dysautonomia; tissue-specific expression of a splicing mutation (REVIEW) PMID: 12102458
    17. Tissue-specific reduction in splicing efficiency of this protein is due to the major mutation associated with familial dysautonomia. PMID: 12577200
    18. The study results suggest that the polymorphisms in the coding region of the IKAP gene are unlikely to contribute to atopic disease risk in the Czech population. PMID: 12774215
    19. whereas IKBKAP (Elongator) is recruited to both target and nontarget genes, only target genes display histone H3 hypoacetylation and progressively lower RNAPII density through the coding region in familial dysautonomia cells PMID: 16713582
    20. Neurodevelopmental disease familial dysautonomia (FD)caused by a single-base change in the 5' splice site (5'ss) of intron 20 in the IKBKAP gene (c.2204+6T>C). PMID: 16964593
    21. investigated the nature of the FD splicing defect and the mechanism by which kinetin improves exon inclusion PMID: 17206408
    22. IKAP/hELP1 may play a role in oligodendrocyte differentiation and/or myelin formation. PMID: 17591626
    23. description of a humanized IKBKAP transgenic mouse that models a tissue-specific human splicing defect PMID: 17644305
    24. IKBKAP may have a role in familial dysautonomia PMID: 18091349
    25. Evidence for the role of the cytosolic interactions of IKAP in cell adhesion and migration, and support the notion that cell-motility deficiencies could contribute to familial dysautonomia. PMID: 18303054
    26. IKAP is crucial for both vascular and neural development during embryogenesis and that protein function is conserved between mouse and human. PMID: 19015235

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  • 相關疾病:
    Neuropathy, hereditary sensory and autonomic, 3 (HSAN3)
  • 亞細胞定位:
    Cytoplasm. Nucleus.
  • 蛋白家族:
    ELP1/IKA1 family
  • 數據庫鏈接:

    HGNC: 5959

    OMIM: 223900

    KEGG: hsa:8518

    STRING: 9606.ENSP00000363779

    UniGene: Hs.494738



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