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  4. Recombinant Human TAR DNA-binding protein 43 (TARDBP)

Recombinant Human TAR DNA-binding protein 43 (TARDBP)

In Stock
  • 中文名稱：
    人TARDBP重組蛋白
  • 貨號：
    CSB-EP023129HU(A4)
  • 規(guī)格：
    ￥2340
  • 圖片：
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他：

產(chǎn)品詳情

  • 純度：
    Greater than 85% as determined by SDS-PAGE.
  • 基因名：
  • Uniprot No.：
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Full Length
  • 來源：
    E.coli
  • 分子量：
    44.9 kDa
  • 表達區(qū)域：
    1-414aa
  • 氨基酸序列
    MSEYIRVTEDENDEPIEIPSEDDGTVLLSTVTAQFPGACGLRYRNPVSQCMRGVRLVEGILHAPDAGWGNLVYVVNYPKDNKRKMDETDASSAVKVKRAVQKTSDLIVLGLPWKTTEQDLKEYFSTFGEVLMVQVKKDLKTGHSKGFGFVRFTEYETQVKVMSQRHMIDGRWCDCKLPNSKQSQDEPLRSRKVFVGRCTEDMTEDELREFFSQYGDVMDVFIPKPFRAFAFVTFADDQIAQSLCGEDLIIKGISVHISNAEPKHNSNRQLERSGRFGGNPGGFGNQGGFGNSRGGGAGLGNNQGSNMGGGMNFGAFSINPAMMAAAQAALQSSWGMMGMLASQQNQSGPSGNNQNQGNMQREPNQAFGSGNNSYSGSNSGAAIGWGSASNAGSGSGFNGGFGSSMDSKSSGWGM
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白標(biāo)簽：
    Tag-Free
  • 產(chǎn)品提供形式：
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 緩沖液：
    If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
  • 復(fù)溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質(zhì)期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期：
    3-7 business days
  • 注意事項：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA：
    Please contact us to get it.

產(chǎn)品評價

靶點詳情

  • 功能：
    RNA-binding protein that is involved in various steps of RNA biogenesis and processing. Preferentially binds, via its two RNA recognition motifs RRM1 and RRM2, to GU-repeats on RNA molecules predominantly localized within long introns and in the 3'UTR of mRNAs. In turn, regulates the splicing of many non-coding and protein-coding RNAs including proteins involved in neuronal survival, as well as mRNAs that encode proteins relevant for neurodegenerative diseases. Plays a role in maintaining mitochondrial homeostasis by regulating the processing of mitochondrial transcripts. Regulates also mRNA stability by recruiting CNOT7/CAF1 deadenylase on mRNA 3'UTR leading to poly(A) tail deadenylation and thus shortening. In response to oxidative insult, associates with stalled ribosomes localized to stress granules (SGs) and contributes to cell survival. Participates also in the normal skeletal muscle formation and regeneration, forming cytoplasmic myo-granules and binding mRNAs that encode sarcomeric proteins. Plays a role in the maintenance of the circadian clock periodicity via stabilization of the CRY1 and CRY2 proteins in a FBXL3-dependent manner. Negatively regulates the expression of CDK6. Regulates the expression of HDAC6, ATG7 and VCP in a PPIA/CYPA-dependent manner.
  • 基因功能參考文獻：
    1. TDP-43 deposition leads to targeted RNA instability in amyotrophic lateral sclerosis and frontotemporal dementia PMID: 30030424
    2. CHCHD10 mutations have a role in cytoplasmic TDP-43 accumulation and synaptic integrity PMID: 28585542
    3. Study confirms the high expression of hTDP-43 in the CNS, increased microgliosis and motor deficits, exhibiting further prominent ALS/FTLD pathologies, such as cytoplasmic and insoluble TDP-43 in TAR6/6 mice. This model represents not only pathological TDP-43 expression but also disease-relevant posttranslational changes. PMID: 29787578
    4. The relevance of contact-independent cell-to-cell transfer of TDP-43 and SOD1 in amyotrophic lateral sclerosis. PMID: 28711596
    5. Findings highlight that the phosphatase regulator, GADD34, also functions as a kinase scaffold in response to chronic oxidative stress and recruits CK1 and oxidized TDP-43 to facilitate its phosphorylation, as seen in TDP-43 proteinopathies. PMID: 29109149
    6. We identified impaired RNA metabolism, secondary to TDP-43 loss of function, as a possible pathological mechanism of HSPB8 toxicity, leading to muscle and nerve degeneration PMID: 29029362
    7. the introduction of SOD1(G93A) and TDP43(A315T), established Amyotrophic lateral sclerosis (ALS)-related mutations, changed the subcellular expression and localization of RNAs within the neurons, showing a spatial specificity to either the soma or the axon. Altogether, we provide here the first combined inclusive profile of mRNA and miRNA expression in two ALS models at the subcellular level PMID: 28300211
    8. These data provide structural detail for the established mechanistic role of the well-folded TDP-43 NTD in splicing and link this function to liquid-liquid phase separation. PMID: 29438978
    9. TDP43 alters most splicing events with splicing factor SRSF3 in triple-negative breast cancer. PMID: 29581274
    10. Of the whole cohort of patients with Motor Neuron Disease and Frontotemporal Dementia, 1 patient harboured a mutation in the TAR DNA-binding protein (TARDBP) gene. PMID: 29886477
    11. Study demonstrated TDP-43/pTDP-43 deposition in skin nerves in ALS patients. Although the mechanisms underlying TDP-43 in ALS are currently unknown, its detection is of interest, and the deposition may occur not only in ALS but also during the aging process which is based on observations of the present study. PMID: 29804146
    12. Both ALS and FTD patients presented with higher TDP-43 and tauT levels compared to the control group. The combination of biomarkers in the form of the TDP-43 x tauT / tauP-181 formula achieved the best discrimination between ALS or FTD and controls, with sensitivities and specificities >0.8. PMID: 28848086
    13. TDP-43 turnover and toxicity depend in part upon the endocytosis pathway. TDP-43 inhibits endocytosis, and co-localizes strongly with endocytic proteins, including in amyotrophic lateral sclerosis patient tissue. PMID: 29233983
    14. more selective group of neurons appears to be affected in frontotemporal lobar degeneration (FTLD)-TDP and FTLD-FUS than in FTLD-tau PMID: 28984110
    15. study found a high frequency of the TARDBP p.M337 V mutation in familial amyotrophic lateral sclerosis (ALS) in south-eastern China; the TARDBP-linked ALS patients showed a benign disease course and prolonged survival PMID: 29621978
    16. describe here two cases of apparently sporadic amyotrophic lateral sclerosis associated with mutations, respectively, in SOD1 and TARDP genes PMID: 27494151
    17. Study indicates that there are at least two common patterns of TDP-43 and tau protein misfolding in human brain aging. In patients lacking substantial Alzheimer's disease pathology, cerebral age-related TDP-43 with sclerosis (CARTS) cases tend to have tau neurofibrillary tangles in the hippocampal dentate granule neurons, providing a potential proxy indicator of CARTS. PMID: 28281308
    18. Depletion of TAF15, FUS and TDP-43 in human-induced pluripotent stem cell-derived motor neurons affects different genes. PMID: 27378374
    19. TDP-43 mislocalisation into axons precedes cell death in cortical neurons, and that cytoskeletal structure and function is impaired by expression of either TDP-43 wild-type or mutant constructs in vitro. PMID: 29787572
    20. TDP-43 impairs the induction of multiple key stress genes required to protect from disease by reducing the recruitment of the chromatin remodeler Chd1 to chromatin. PMID: 29153328
    21. The mutation of TARDBP caused amyotrophic lateral sclerosis PMID: 29478603
    22. Cytoplasmic TDP-43 mislocalization and aggregation is a pathological hallmark of amyotrophic lateral sclerosis and frontotemporal lobar degeneration. TDP-43 is an RNA-binding protein with a prion-like domain that promotes TDP-43 misfolding. [review] PMID: 27920024
    23. SOD1 mutations were present in 20% of familial amyotrophic lateral sclerosis (ALS) patients and 1.9% of sporadic ALS patients, while FUS mutations were responsible for 13.3% of familial ALS cases, and TARDBP mutations were rare in either familial or sporadic ALS cases. PMID: 27604643
    24. an alpha-helical component in the centre (residues 320-340) of the C-terminal domain is related to the protein's self-association and LLPS. Systematically analysing ALS-related TDP-43 mutants (G298S, M337V, and Q331K) in different buffer conditions at different temperatures, we prove that this phase separation is driven by hydrophobic interactions but is inhibited by electrostatic repulsion. PMID: 28988034
    25. the present study did not demonstrate oxidative phosphorylation defects in TDP-43 mutants PMID: 28482850
    26. both FUS and TDP43 colocalize with active RNA polymerase II at sites of DNA damage along with the DNA damage repair protein, BRCA1, and FUS and TDP43 participate in the prevention or repair of R loop-associated DNA damage, a manifestation of aberrant transcription and/or RNA processing PMID: 27849576
    27. The data of this study suggest that TDP-43 pathology is associated with age and exacerbated by the presence of concomitant Alzheimer's disease pathology. PMID: 27495267
    28. The A382T mutation in TARDBP caused a reduction in the ability of cells to respond to stress through loss of TDP-43 function in stress granule nucleation. The pathogenetic action revealed in study model does not seem to be mediated by changes in the localization of the TDP-43 protein. PMID: 28172957
    29. TDP-43 competes with other splicing factors for binding to cryptic exons and can repress cryptic exon inclusion. PMID: 28549443
    30. Study shows that TDP-43 is deposited in the olfactory bulb in Alzheimer's disease, albeit of low frequency. The deposition appears to be a late occurrence compared to TDP-43 deposition in other brain regions. PMID: 26810591
    31. ALS-mutant linked TDP-43 mutations expressed at moderate levels in a pattern mimicking endogenous TDP-43 also cause toxicity in a non-cell autonomous manner. Eliminating mutant TDP-43Q331K synthesis in a proportion of motor neurons delayed disease onset, reduced aberrant nuclear morphology in those neurons at early disease stages, and almost eliminated age-dependent accelerated death of those motor neurons. PMID: 28357566
    32. Study reports that cryptic exon incorporation occurred not only in Alzheimer' disease brains exhibiting TDP-43 pathology, but also in neurons lacking cytoplasmic inclusion but exhibiting nuclear clearance of TDP-43. PMID: 28332094
    33. Acetylation of the protein triggers TDP-43 pathology in cultured cells and mouse skeletal muscle, which can be cleared through an HSF1-dependent chaperone mechanism that disaggregates the protein. PMID: 28724966
    34. These studies showed that physiological oligomerization of TDP-43 is mediated through its N-terminal domain, which forms functional and dynamic oligomers antagonizing pathologic aggregation. PMID: 28663553
    35. Expression of PFN1 mutants induces accumulation of TDP-43, and promotes conversion of normal TDP-43 into an abnormal form. These results provide new insight into the mechanisms of TDP-43 proteinopathies and other diseases associated with amyloid-like protein deposition. PMID: 27432186
    36. Study reports the altered expression and/or mislocalization of the TAR-DNA binding protein 43 (TDP-43) in both niemann-pick disease type C mouse and in a human neuronal model of the disease. Results extend the importance of the role of TDP-43 in neurodegenerative disease and further highlight the need to prioritize the targeting of this protein to develop novel therapeutic strategies. PMID: 27193329
    37. This study demonstrated that increased rates of TDP-43-associated hippocampal atrophy might occur at least 10 years before death in patient with Alzheimer disease. PMID: 28919059
    38. Authors observed impaired levels of glutathione (downstream Nrf2 antioxidant) in TDP-43M337V patient fibroblasts and astrocyte cultures from TDP-43Q331K mice, indicative of elevated oxidative stress and failure of some upregulated antioxidant genes to be translated into protein. PMID: 28334913
    39. removing the human orthologs of Hrb27c (DAZAP1) in human neuronal cell lines can correct several pre-mRNA splicing events altered by TDP-43 depletion PMID: 28575377
    40. TDP-43 suppressed tau expression by promoting its mRNA instability through the UG repeats of its 3-UTR. The C-terminal region of TDP-43 was required for this function.The level of TDP-43, which is decreased in AD brains, was found to correlate negatively with the tau level in human brain. PMID: 28335005
    41. Amyotrophic lateral sclerosis mutations disrupt phase separation mediated by alpha-helical structure in the TDP-43 low-complexity C-terminal domain. PMID: 27545621
    42. we demonstrated cytoplasmic TDP-43 aggregate formation in neuronal and glial cells following adenoviral transduction of WT and CTF TDP-43 under MG-132 treatment. These TDP-43 aggregates were phosphorylated and ubiquitinated and consisted of electron-dense granules. PMID: 28599005
    43. emphasize the importance of distinguishing cerebral age-related TDP-43 with sclerosis from late-onset frontotemporal lobar degeneration with TDP-43 pathology and from advanced Alzheimer disease with TDP-43 pathology PMID: 28467211
    44. Mutant and wild type human TDP-43 replacing the endogenous Drosophila gene reveals phosphorylation and ubiquitination in mutant lines in the absence of viability or lifespan defects. PMID: 28686708
    45. The study establishes a functional/physical partnership between FMRP and TDP-43 that mechanistically links several neurodevelopmental disorders and neurodegenerative diseases. PMID: 27518042
    46. By silencing TDP-43, authors saw significant inhibition of cell proliferation and metastasis in A375 and WM451 cells. TDP-43 knockdown could suppress glucose transporter type-4 (GLUT4) expression and reduce glucose uptake. PMID: 27786596
    47. The present study, based on 15 cases staged for pTDP-43 pathology, reports the finding that pathologically altered TDP-43 in Betz cells reacts differently than that in bulbar or spinal alpha-motoneurons. The major differences between the two types of histological profiles are discussed within the context of their possible consequences and implications for the potential further progression or spread of the pTDP-43 lesions. PMID: 27757524
    48. This study have shown that TDP-43-positive cytoplasmic inclusions were frequently found in the amygdala of pathologically and genetically confirmed cases of Frontotemporal Lobar Degeneration and Motor Neuron Disease. PMID: 28859337
    49. Results indicated that a range of disease specific TDP-43 variants are generated in amyotrophic lateral sclerosis patients with different variants being generated in sporadic and familial cases. PMID: 28122516
    50. heterogeneous structural reorganization and decreased stability of the truncated RRM2 domain PMID: 28793209

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  • 相關(guān)疾?。?/div>
    Amyotrophic lateral sclerosis 10 (ALS10)
  • 亞細(xì)胞定位：
    Nucleus. Cytoplasm. Cytoplasm, Stress granule.
  • 組織特異性：
    Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 11571

    OMIM: 605078

    KEGG: hsa:23435

    STRING: 9606.ENSP00000240185

    UniGene: Hs.300624