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  4. Recombinant Human Nebulin (NEB), partial

Recombinant Human Nebulin (NEB), partial

  • 中文名稱：
    人NEB重組蛋白
  • 貨號：
    CSB-YP324989HU
  • 規(guī)格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    人NEB重組蛋白
  • 貨號：
    CSB-EP324989HU
  • 規(guī)格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    人NEB重組蛋白
  • 貨號：
    CSB-EP324989HU-B
  • 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NEB重組蛋白
  • 貨號：
    CSB-BP324989HU
  • 規(guī)格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    人NEB重組蛋白
  • 貨號：
    CSB-MP324989HU
  • 規(guī)格：
  • 來源：
    Mammalian cell
  • 其他：

產品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    NEB
  • Uniprot No.：
  • 別名：
    DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; NEB 177D; NEB; NEB177D; NEBU_HUMAN; Nebulin; NEM 2; NEM2
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Partial
  • 蛋白標簽：
    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.

產品評價

靶點詳情

  • 功能：
    This giant muscle protein may be involved in maintaining the structural integrity of sarcomeres and the membrane system associated with the myofibrils. Binds and stabilize F-actin.
  • 基因功能參考文獻：
    1. NEB gene should be included in genetic panels for fetal akinesia/arthrogryposis multiplex congenital cases. PMID: 27933661
    2. We examined a Chinese strabismus pedigree with the parents unaffected and 2 offspring affected. Whole-exome sequencing and bioinformatics filtering identified 2 variants including Abelson helper integration site 1 (AHI1) gene and nebulin (NEB) gene. c.A914G mutation was found in nebulin (NEB) gene. PMID: 28391287
    3. Shorter than normal thin filament length contributes to the impaired force generation in patients with thin filament myopathy, but only in those who harbor specific mutations in NEB or ACTA1. PMID: 27074222
    4. New NEB mutations were found in 8 of 10 patients with nemaline myopathy. PMID: 27105866
    5. recurrent NEB TRI copy number variation was found in 13% of the families with nemaline myopathy and in 10% of the controls. PMID: 26197980
    6. Prominent foot-drop was associated with NEB gene mutations in three patients with core-rod myopathy. PMID: 26403434
    7. Identified two novel, compound-heterozygous variants in the nebulin gene after a 30 year clinical history, which cause a classical childhood type of nemaline myopathy. PMID: 25740301
    8. Since the patients are characterized by generalized muscle weakness together with neurodevelopmental phenotypes, it is suggested that NEB mutations could manifest more diverse phenotypes than those previously described. PMID: 25296583
    9. indicates that nebulin tolerates substantial changes in its amino acid sequence, providing an explanation as to why variants in such a large gene result in relatively rare disorders PMID: 25205138
    10. Mutations in NEB gene is associated with stress fracture. PMID: 25023003
    11. This study demonistrated that Muscle histopathology in nebulin-related nemaline myopathy: ultrastrastructural findings correlated to disease severity. PMID: 24725366
    12. We identify the SH3 domains of nebulin and nebulette as novel ligands of proline-rich regions of Xin and XIRP2 PMID: 23985323
    13. Data suggest that for some filament-forming desmin mutants, the molecular etiology of desminopathy results from subtle deficiencies in their association with nebulin, a major actin-binding filament protein of striated muscle. PMID: 23615443
    14. A nebulin-based nemaline myopathy model is characterized in transgenic mice following deletion of exon 55 in nebulin. PMID: 23715096
    15. Multiple isoforms of nebulin expressed in skeletal muscle and brain may have a role as actin filament stabilizer or length regulator in neurons of the human brain PMID: 22941678
    16. Ultrasonographic findings suggestive of a myopathy and a carrier state for the NEB exon 55 deletion in one of the parents should trigger a thorough investigation for nemaline. PMID: 22367672
    17. distal nemaline myopathy caused by four different compound heterozygous nebulin mutations PMID: 21724397
    18. The mechanics as well as the X-ray diffraction patterns of human membrane-permeabilized single muscle fibers expressing nebulin mutations are reported. PMID: 21350120
    19. Data revealed markedly reduced nebulin protein levels in muscle from nemaline myopathy patients, whereas levels of other thin filament-based proteins were not significantly altered. PMID: 19944167
    20. Nebulin regulates thin filament architecture by a mechanism that includes stabilizing the filaments and preventing actin depolymerization. PMID: 20498015
    21. SH3 domain of nebulin binds selectively to type II peptides: theoretical prediction and experimental validation PMID: 11851340
    22. Lack of the C-terminal domain of nebulin in a patient with nemaline myopathy. PMID: 11994971
    23. nebulin colocalizes with desmin in a Z-line-associated, striated pattern, thereby forming a lateral linkage system which contributes to maintain adjacent Z-lines in register as shown by immunofluorescence studies PMID: 12064939
    24. Mutations caused absence of the C-terminal part of nebulin resulting in severe congenital form of nemaline myopathy. PMID: 12207937
    25. Nebulin is targeted and oriented through titin and myopalladin signaling during sarcomere assembly PMID: 12482578
    26. 45 novel mutations within the nubulin gene are associated with nemaline myopathy. PMID: 16917880
    27. These data suggest that N-terminal superrepeat nebulin modules are incapable of supporting interactions with the cardiac myofilaments; whereas the C-terminal nebulin modules can. PMID: 17275809
    28. These data suggest a model in which archvillin attaches directly to the Z-line of skeletal muscle through an interaction with the nebulin C-terminus. PMID: 18639526
    29. In all but two of eight homozygous patients with nebulin mutations, the clinical picture was more severe than in typical nemaline myopathy. PMID: 19232495
    30. Nebulin is thought to serve as both a length-regulating protein ruler and calcium/calmodulin-mediated regulatory protein on the thin filaments of the skeletal muscle sarcomere. PMID: 11425319

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  • 相關疾病：
    Nemaline myopathy 2 (NEM2)
  • 亞細胞定位：
    Cytoplasm, myofibril, sarcomere. Cytoplasm, cytoskeleton.
  • 組織特異性：
    Muscle specific. Located in the thin filament of striated muscle.
  • 數據庫鏈接：

    HGNC: 7720

    OMIM: 161650

    KEGG: hsa:4703

    UniGene: Hs.588655



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