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  4. Recombinant Human Inner nuclear membrane protein Man1 (LEMD3), partial

Recombinant Human Inner nuclear membrane protein Man1 (LEMD3), partial

  • 中文名稱：
    人LEMD3重組蛋白
  • 貨號：
    CSB-YP896701HU
  • 說明書：
  • 規(guī)格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    人LEMD3重組蛋白
  • 貨號：
    CSB-EP896701HU
  • 說明書：
  • 規(guī)格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    人LEMD3重組蛋白
  • 貨號：
    CSB-EP896701HU-B
  • 說明書：
  • 規(guī)格：
  • 來源：
    E.coli
  • 共軛：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名稱：
    人LEMD3重組蛋白
  • 貨號：
    CSB-BP896701HU
  • 說明書：
  • 規(guī)格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    人LEMD3重組蛋白
  • 貨號：
    CSB-MP896701HU
  • 說明書：
  • 規(guī)格：
  • 來源：
    Mammalian cell
  • 其他：

產(chǎn)品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    LEMD3
  • Uniprot No.：
  • 別名：
    Inner nuclear membrane protein Man1; LEM domain containing protein 3 ; LEM domain-containing protein 3; LEMD3; MAN1_HUMAN
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Partial
  • 蛋白標(biāo)簽：
    Tag?type?will?be?determined?during?the?manufacturing?process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 產(chǎn)品提供形式：
    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.
  • 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：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

產(chǎn)品評價

靶點詳情

  • 功能：
    Can function as a specific repressor of TGF-beta, activin, and BMP signaling through its interaction with the R-SMAD proteins. Antagonizes TGF-beta-induced cell proliferation arrest.
  • 基因功能參考文獻(xiàn)：
    1. studies demonstrated that lower levels of MAN1 in differentiating MSC are associated with higher osteogenesis and lower adipogenesis. High levels of MAN1 only affected adipogenesis. PMID: 28449239
    2. LAMD3 Y871X mutation is associated with osteopoikilosis. PMID: 26694706
    3. Letter/Case-Report: novel frameshift mutation in RNA recognition motif of LEMD3 in patient with Buschke-Ollendorff syndrome. PMID: 26711937
    4. A novel mutation in LEMD3 splice site results in Buschke-Ollendorff syndrome. PMID: 26135202
    5. Data indicate that the inner nuclear membrane protein MAN1 directly binds the transcription activator BMAL1 promoter and enhances its transcription. PMID: 25182847
    6. a nuclear envelope-localized mechanism of inactivating TGF-beta signaling in which MAN1 competes with transcription factors for binding to Smad2 and Smad3 and facilitates their dephosphorylation by PPM1A. PMID: 23779087
    7. The absence of direct binding of BAF to MAN1-C eliminates disruption of this interaction as the cause of the premature aging phenotype. PMID: 21966431
    8. Genetic analyses of three generations of a family with Buschke-Ollendorff syndrome having a variable phenotype showed a novel c.2203C>T nonsense mutation at the LEMD3 locus. The mutation induced a change in the 735 arginine codon to a stop codon. PMID: 20678097
    9. Buschke-Ollendorff syndrome in a three-generation family: influence of a novel LEMD3 mutation to tropoelastin expression. PMID: 20732851
    10. We found a novel c.2203C > T (p.R735X) mutation in exon 9 of LEMD3, resulting in a premature stop codon at amino acid position 735. PMID: 20618940
    11. Absence of LEMD3 mutation in the exons and splice sites of a family with BOS suggests that there is genetic heterogeneity for this disorder. PMID: 20083694
    12. The C-terminal domain of human MAN1 binds to Smad2 and Smad3 and antagonizes signaling by transforming growth factor-beta PMID: 15601644
    13. Overexpression results in inhibition of R-Smad phosphorylation, heterodimerization with Smad4, and nuclear translocation PMID: 15647271
    14. Data describe the direct binding of the nuclear membrane protein MAN1 to emerin in vitro. PMID: 15681850
    15. germline LEMD3 mutations are rare in sporadic patients with isolated melorheostosis PMID: 16470551
    16. MAN1 binds simultaneously to R-Smads and their targeted DNA sequences PMID: 16648637
    17. novel mutation associated with familial cutaneous collagenomas PMID: 17223882
    18. novel heterozygous splice-site mutation in a Japanese kindred with Buschke-Ollendorff syndrome PMID: 17505164
    19. The LEMD3 mutation reported was clearly the cause of osteopoikilosis in the two families but its relationship to melorheostosis in one of the family members is still unclear. PMID: 17622481
    20. Identified two mutations in the two cases of Buschke-Ollendorff syndrome. The mutation (c.2564G>A) is novel. The present study supports the general conclusion that LEMD3 mutations do not contribute to isolated sporadic melorheostosis. PMID: 19438932
    21. Binds to Smad2 and Smad3 and antagonizes signaling by transforming growth factor-beta PMID: 15601644

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  • 相關(guān)疾病：
    Buschke-Ollendorff syndrome (BOS)
  • 亞細(xì)胞定位：
    Nucleus inner membrane; Multi-pass membrane protein.
  • 組織特異性：
    Heart, brain, placenta, lung, liver and skeletal muscle.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 28887

    OMIM: 166700

    KEGG: hsa:23592

    STRING: 9606.ENSP00000308369

    UniGene: Hs.744150



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