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MATR3 Antibody

  • 货号：
    CSB-PA125471
  • 规格：
    ￥1100
  • 图片：
    • Gel: 6%SDS-PAGE, Lysate: 40 μg, Lane 1-6: 293T cells, K562 cells, hela cells, 231 cells, Jurkat cells, NIH/3T3 cells, Primary antibody: CSB-PA125471(MATR3 Antibody) at dilution 1/500, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 3 seconds
  • 其他：

产品详情

  • Uniprot No.：
    P43243
  • 基因名：
    MATR3
  • 别名：
    ALS21 antibody; KIAA0723 antibody; Matr3 antibody; MATR3_HUMAN antibody; Matrin-3 antibody; Matrin3 antibody; MPD2 antibody; VCPDM antibody; Vocal cord and pharyngeal weakness with distal myopathy antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Fusion protein of Human MATR3
  • 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,WB
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    WB 1:500-1:2000
  • 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：
    May play a role in transcription or may interact with other nuclear matrix proteins to form the internal fibrogranular network. In association with the SFPQ-NONO heteromer may play a role in nuclear retention of defective RNAs. Plays a role in the regulation of DNA virus-mediated innate immune response by assembling into the HDP-RNP complex, a complex that serves as a platform for IRF3 phosphorylation and subsequent innate immune response activation through the cGAS-STING pathway. May bind to specific miRNA hairpins.
  • 基因功能参考文献：
    1. Depletion of SAFB1 reduced FUS's localization to chromatin-bound fraction and splicing activity, suggesting SAFB1 could tether FUS to chromatin compartment thorough N-terminal DNA-binding motif. Moreover, FUS interacts with another nuclear matrix-associated protein, Matrin3. PMID: 27731383
    2. Using a novel RNA pulldown approach that utilized endogenous S1-tagged PINCR (a p53-regulated long noncoding RNA), the s show that PINCR associates with the enhancer region of these genes by binding to RNA-binding protein Matrin 3 that, in turn, associates with p53. PMID: 28580901
    3. Matrin3 physically interacts with intronic pyrimidine-rich sequences and controls alternative splicing. PMID: 28695676
    4. It may not be a common genetic factor in Chinese amyotrophic lateral sclerosis patients. PMID: 26708275
    5. Its mutations strengthens the role of RNA metabolism in amyotrophic lateral sclerosis etiology. PMID: 26493020
    6. A missense mutation in MATR3 was identified in myopathy patients undergoing a needle electromyography. PMID: 26899464
    7. Our findings indicate that mutations in Matrin 3 that are associated with ALS and myopathy do not dramatically alter the normal localization of the protein or readily induce inclusion formation. PMID: 26528920
    8. Three-dimensional mapping of the lamin A-matrin-3 interface showed that the LMNA truncating mutation Delta303, which lacks the matrin-3 binding domain, was associated with an increased distance between lamin A and matrin-3. PMID: 25948554
    9. Suppressing Matrin 3 powers a heightened and broader ZAP restriction of HIV-1 gene expression. PMID: 26129669
    10. MATR3 mutation is identified to be a possible cause of amyotrophic lateral sclerosis. PMID: 25771394
    11. No mutations were identified, indicating that MATR3 mutations are not a common cause of Amyotrophic lateral sclerosis in Australian familial cases with predominately European ancestry PMID: 25523636
    12. MATR3 gene disruption is associated with bicuspid aortic valve, aortic coarctation and patent ductus arteriosus. PMID: 25574029
    13. Mutations in MATR3 are rare in French familial ALS and ALS with FTLD patients. PMID: 25158920
    14. This study shows a high risk of abnormal respiratory function with progressive worsening in MATR3 myopathy. PMID: 25677933
    15. Nuclear matrix protein Matrin3 regulates alternative splicing and forms overlapping regulatory networks with PTB. PMID: 25599992
    16. the pY RNA1-s2/Matr3 interaction could play a role in vision PMID: 24558381
    17. Sixteen patients from 6 families with late onset distal myopathy associated with the p.S85C MATR3 mutation were characterized. PMID: 25154462
    18. This study identified mutations in MATR3 in ALS kindreds. also observed MATR3 pathology in ALS-affected spinal cords with and without MATR3 mutations. PMID: 24686783
    19. Data suggest that the cellular level of MATR3, known to be highly regulated, modulates the stability of a group of gene transcripts. PMID: 21858232
    20. Rev needs MATR3 to promote the cytoplasmic accumulation and translation of unspliced RRE-containing mRNA. PMID: 21771348
    21. MATR3 binds viral RNA and is required for the Rev/RRE mediated nuclear export of unspliced HIV-1 RNAs. PMID: 21771346
    22. Matrin 3 binds Rev RNA to stabilize HIV-1 transcripts leading to increased cytoplasmic expression. PMID: 21771347
    23. MATR3 to be a novel ATM target in response to DNA damage. PMID: 20421735
    24. matrin 3 plays a significant role in controlling cell growth and proliferation PMID: 21182838
    25. study of the association of matr3 with chromosome territories and identification of potential interacting proteins PMID: 19562669
    26. Data show that the nuclear matrix protein matrin 3, cytoskeletal motor protein HMP, and the circadian clock protein lark were significantly decreased in fetal Down syndrome brain. PMID: 12469345
    27. These results suggest that the functions of matrin 3 could be regulated by both Ca(2+)-dependent interaction with calmodulin and caspase-mediated cleavage. PMID: 17658460
    28. the spatial proximities among a constellation of functionally related sites that are found within euchromatic regions of the cell nucleus including: HP1gamma, RNA polymerase II, matrin 3, and SAF-A sites PMID: 18618731
    29. nonconservative S85C missense mutation in vocal cord and pharyngeal weakness with distal myopathy PMID: 19344878

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  • 相关疾病：
    Amyotrophic lateral sclerosis 21 (ALS21)
  • 亚细胞定位：
    Nucleus matrix.
  • 数据库链接：

    HGNC: 6912

    OMIM: 164015

    KEGG: hsa:9782

    STRING: 9606.ENSP00000354346

    UniGene: Hs.268939