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COL4A3BP Antibody

  • 货号：
    CSB-PA223164
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA223164(COL4A3BP Antibody) at dilution 1/50, on the right is treated with fusion protein. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human gastric cancer tissue using CSB-PA223164(COL4A3BP Antibody) at dilution 1/50, on the right is treated with fusion protein. (Original magnification: ×200)
  • 其他：

产品详情

  • Uniprot No.：
    Q9Y5P4
  • 基因名：
    COL4A3BP
  • 别名：
    Alpha 3 type IV collagen binding protein antibody; C43BP_HUMAN antibody; Ceramide transfer protein antibody; Ceramide transporter antibody; CERT antibody; Cert protein antibody; CERTL antibody; col4a3bp antibody; Collagen type IV alpha 3 (Goodpasture antigen) binding protein antibody; Collagen type IV alpha 3 binding protein antibody; Collagen type IV alpha-3-binding protein antibody; FLJ20597 antibody; Goodpasture antigen binding protein antibody; Goodpasture antigen-binding protein antibody; GPBP antibody; hCERT antibody; Lipid transfer protein CERTL antibody; Procollagen type IV alpha 3 (Goodpasture antigen) binding protein antibody; StAR related lipid transfer (START) domain containing 11 antibody; StAR related lipid transfer protein 11 antibody; StAR-related lipid transfer protein 11 antibody; StARD 11 antibody; StARD11 antibody; START domain containing 11 antibody; START domain containing protein 11 antibody; START domain-containing protein 11 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Fusion protein of Human COL4A3BP
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    IHC 1:50-1:200
  • 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：
    Shelters ceramides and diacylglycerol lipids inside its START domain and mediates the intracellular trafficking of ceramides and diacylglycerol lipids in a non-vesicular manner.
  • 基因功能参考文献：
    1. Importance of Endoplasmic reticulum to Golgi ceramide traffic to preserve muscle cell insulin signaling, CERT as a major actor in this process. PMID: 29759974
    2. Data suggest that isolated PH and START domains of CERT interact with each other; START domain binds to PH domain at same site for PtdIns[4]P-binding; mutations disrupting PH-START interaction increase both PtdIns[4]P-binding affinity and ceramide transfer activity. (PH domain = N-terminal pleckstrin homology domain; START domain = StAR-related lipid transfer domain; PtdIns[4]P = phosphatidylinositol 4-phosphate) PMID: 28652409
    3. Cells expressing mitoCERT import ceramides into mitochondria and undergo Bax-dependent apoptosis. PMID: 27888218
    4. phosphorylation of CERT at the FFAT motif-adjacent serine affects its affinity for VAP, which may regulate the inter-organelle trafficking of ceramide in response to the perturbation of cellular sphingomyelin and/or other sphingolipids PMID: 24569996
    5. Both CERT isoforms, when immobilized, bind the globular head region of C1q and initiate the classical complement pathway. C1q binds endogenous CERTL on the surface of apoptotic cells. PMID: 24395916
    6. Structural basis for the Golgi association by the pleckstrin homology domain of the ceramide trafficking protein (CERT) PMID: 22869376
    7. CERT is at a convergence point of non-vesicular and vesicular transport processes and plays a central role within the PKD signaling network at the Golgi complex. (Review) PMID: 22226883
    8. Loss of the ceramide transfer protein augments EGF receptor signaling in breast cancer. PMID: 22472120
    9. The START domain in GPBP is important for this interaction. SAP and GPBP form complexes in blood and partly colocalize in amyloid plaques from Alzheimer disease patients. PMID: 22396542
    10. This review is focused on ceramide transfer protein (CERT) as a major regulator of ceramide flux in the cell. [review] PMID: 21707482
    11. during cellular stress, disassembly of Golgi structure together with inactivation of CERT by caspases causes a reduction in ceramide trafficking and sphingomyelin synthesis, and could contribute to the cellular response to pro-apoptotic stress PMID: 22129459
    12. the presence of IncD, CERT, VAPA/B, and potentially additional host and/or bacterial factors, at points of contact between the ER and the inclusion membrane provides a specialized microenvironment favorable to bacterial development. PMID: 21731489
    13. We hypothesize that sphingomyelin acquired by CERT-dependent transport of ceramide and subsequent conversion to SM is necessary for C. trachomatis replication whereas SM acquired by GBF1-dependent pathway is essential for inclusion growth and stability. PMID: 21909260
    14. GPBP directs myofibril formation through interaction with intracellular downstream effector 130-kDa GPBP-interacting protein PMID: 21832087
    15. PKD negatively regulates HCV secretion/release by attenuating OSBP and CERT functions by phosphorylation inhibition. This study identifies the key role of the Golgi components in the HCV maturation process. PMID: 21285358
    16. the membrane matrix surrounding ceramide, that is ceramide miscibility, is largely affecting the transfer activity of CERT PMID: 20875392
    17. hBVR is a regulator of the TNF-alpha-GPBP-collagen type IV signaling cascade PMID: 20177069
    18. Crystal structures of the CERT START domain in complex with HPAs ((1R,3R)-N-(3-Hydroxy-1-hydroxymethyl-3-phenylpropyl)alkanamide) of varying acyl chain lengths. PMID: 20036255
    19. CERT mediates the intracellular trafficking of ceramide in a non-vesicular manner PMID: 14685229
    20. results indicate that CERT can mediate transfer of various types of ceramides that naturally exist and their close relatives PMID: 15596449
    21. crypticity is a feature of the quaternary structure of two subsets of alpha3alpha4alpha5(IV) NC1 hexamers: autoantibody-reactive M-hexamers containing only monomer subunits and autoantibody-impenetrable D-hexamers composed of dimer and monomer subunits PMID: 15917228
    22. Results identify a human-specific TNF-responsive transcriptional unit that locates GPBP in the signalling cascade of TNF and substantiates previous observations, which independently related TNF and GPBP with human autoimmunity. PMID: 16218959
    23. A clinical evaluation of probands and their relatives of the five families carrying mutations in either the COL4A3 or the COL4A4 gene was carried out to underline the natural history of the autosomal recessive ATS. PMID: 16338941
    24. Regulation of ceramide transport protein by oxysterol binding protein, and VAMP reveals a novel mechanism for ceramide transport and sphingophingomyelin synthesis in the Golgi apparatus. PMID: 16571669
    25. The Golgi-targeting domain and endoplasmic reticulum-interacting FFAT amino acid motif of CERT spatially restrict the random ceramide transfer activity of the START domain in cells. PMID: 16895911
    26. Data show that CERT receives multiple phosphorylations at a serine-repeat motif, a possibe site for casein kinase I, and that the phosphorylation down-regulates the ER-to-Golgi transport of ceramide. PMID: 17442665
    27. Regulation of secretory transport by PKD-mediated phosphorylation of CERT is reported. PMID: 17591919
    28. GPBP regulates GBM collagen organization and its elevated expression causes dissociation and subsequent accumulation of IgA on the GBM. PMID: 17916599
    29. CERT is a physiological substrate of PP2Cepsilon and that dephosphorylation of CERT by PP2Cepsilon may play an important role in the regulation of ceramide trafficking from the ER to the Golgi apparatus. PMID: 18165232
    30. analysis of the mechanism by which CERT can distinguish ceramide from other lipid types yet still recognize multiple species of ceramides PMID: 18184806
    31. healthy individuals have low frequencies of unstimulated alpha3(IV)NC1-reactive T cells with similar specificities to the autoreactive T cells found in patients with Goodpasture disease PMID: 18216317
    32. CERT can attenuate a key metabolic protective mechanism against ceramide-induced apoptosis in keratinocytes PMID: 18411267
    33. Goodpasture antigen-binding protein is a soluble exportable protein that interacts with type IV collagen PMID: 18772132
    34. These results indicate that CKIgamma2 hyperphosphorylates the serine-repeat motif of CERT, thereby inactivating CERT and down-regulating the synthesis of sphingomyelin. PMID: 19005213
    35. the allergen causing the most food-induced anaphylaxis in Italy PMID: 19494524

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  • 相关疾病：
    Mental retardation, autosomal dominant 34 (MRD34)
  • 亚细胞定位：
    Cytoplasm. Golgi apparatus. Endoplasmic reticulum.
  • 组织特异性：
    Widely expressed.
  • 数据库链接：

    HGNC: 2205

    OMIM: 604677

    KEGG: hsa:10087

    STRING: 9606.ENSP00000369862

    UniGene: Hs.270437