Recombinant Human Merlin(NF2)

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Code CSB-EP015741HU
Size US$2466
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  • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

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Product Details

Purity Greater than 85% as determined by SDS-PAGE.
Target Names NF2
Uniprot No. P35240
Research Area Cancer
Alternative Names ACN; BANF; Bilateral acoustic neuroma; MERL_HUMAN; Merlin; Moesin ezrin radixin like protein; Moesin ezrin radizin like; Moesin-ezrin-radixin-like protein; Neurofibromatosis 2; Neurofibromatosis type 2; Neurofibromatosis2; Neurofibromin 2; Neurofibromin-2; Neurofibromin2; NF 2; Nf2; SCH; Schwannomerlin; Schwannomin
Species Homo sapiens (Human)
Source E.coli
Expression Region 1-595aa
Target Protein Sequence MAGAIASRMSFSSLKRKQPKTFTVRIVTMDAEMEFNCEMKWKGKDLFDLVCRTLGLRETWFFGLQYTIKDTVAWLKMDKKVLDHDVSKEEPVTFHFLAKFYPENAEEELVQEITQHLFFLQVKKQILDEKIYCPPEASVLLASYAVQAKYGDYDPSVHKRGFLAQEELLPKRVINLYQMTPEMWEERITAWYAEHRGRARDEAEMEYLKIAQDLEMYGVNYFAIRNKKGTELLLGVDALGLHIYDPENRLTPKISFPWNEIRNISYSDKEFTIKPLDKKIDVFKFNSSKLRVNKLILQLCIGNHDLFMRRRKADSLEVQQMKAQAREEKARKQMERQRLAREKQMREEAERTRDELERRLLQMKEEATMANEALMRSEETADLLAEKAQITEEEAKLLAQKAAEAEQEMQRIKATAIRTEEEKRLMEQKVLEAEVLALKMAEESERRAKEADQLKQDLQEAREAERRAKQKLLEIATKPTYPPMNPIPAPLPPDIPSFNLIGDSLSFDFKDTDMKRLSMEIEKEKVEYMEKSKHLQEQLNELKTEIEALKLKERETALDILHNENSDRGGSSKHNTIKKLTLQSAKSRVAFFEEL
Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
Mol. Weight 73.7 kDa
Protein Length Full Length
Tag Info N-terminal 6xHis-tagged
Form 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.
Buffer If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
Note: If you have any special requirement for the glycerol content, please remark when you place the order.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Reconstitution 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°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting
and FAQs
Protein FAQs
Storage Condition Store at -20°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life 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.
Lead Time Basically, we can dispatch the products out in 3-7 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Notes 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.

Target Data

Function Probable regulator of the Hippo/SWH (Sav/Wts/Hpo) signaling pathway, a signaling pathway that plays a pivotal role in tumor suppression by restricting proliferation and promoting apoptosis. Along with WWC1 can synergistically induce the phosphorylation of LATS1 and LATS2 and can probably function in the regulation of the Hippo/SWH (Sav/Wts/Hpo) signaling pathway. May act as a membrane stabilizing protein. May inhibit PI3 kinase by binding to AGAP2 and impairing its stimulating activity. Suppresses cell proliferation and tumorigenesis by inhibiting the CUL4A-RBX1-DDB1-VprBP/DCAF1 E3 ubiquitin-protein ligase complex.
Gene References into Functions
  1. lipid binding results in the open conformation of neurofibromin 2 and lipid binding is necessary for inhibiting cell proliferation PMID: 29626191
  2. Cell viability results showed that three agents (GSK2126458, Panobinostat, CUDC-907) had the greatest activity across schwannoma and meningioma cell systems, but merlin status did not significantly influence response PMID: 29897904
  3. Merlin loss increased oxidative stress causing aberrant activation of Hedgehog signaling in in vitro. PMID: 28112165
  4. NF2 promoter gene mutations occurred in medulloblastoma (MB) patients. The NF2 mRNA expression was higher in the controls than in patients; however NF2 protein expression was significantly higher in patients than in the controls. NF2 protein was mainly expressed in the nucleus in MB patients, while the NF2 protein was mainly expressed in the cytoplasm in the controls. PMID: 29637450
  5. Study summarizes the current knowledge of molecular events triggered by NF2/merlin inactivation, which lead to the development of mesothelioma and other cancers. Genetic alterations in NF2 that abrogate merlin's functional activity are found in about 40% of malignant mesothelioma (MM), indicating the importance of NF2 inactivation in MM development and progression. [review] PMID: 29587439
  6. Study identified missense NF2 mutations in 1.9% hepatocellular carcinoma (HCC) and 5.3% intrahepatic cholangiocarcinoma (ICC). Allele frequency of NF2 IVS4-39 A/A was significantly higher in HCCs. Also, NF2/Merlin showed a dual role as a tumorigenic gene and tumor-suppressor gene; Merlin was expressed at higher levels in HCC tumors ; while the rate of Merlin upregulation was lower in poorly differentiated ICCs. PMID: 29130106
  7. This study demonstrates that simultaneous inhibition of c-Met and Src signaling in MD-MSCs triggers apoptosis and reveals vulnerable pathways that could be exploited to develop NF2 therapies. PMID: 28775147
  8. The genetic alterations observed in the NF2 gene indicated that spinal schwannomas are associated with genetic alterations also found in other schwannomas and type 2 Neurofibromatosis, which reinforces the etiological role of this gene. PMID: 29599333
  9. Methylation of NF2 and DNMT1 was markedly increased, and miR-152-3p was downregulated in GBM tissues and glioma cells. Both knockdown of DNMT1 and overexpression miR-152-3p showed that demethylation activated the expression of NF2. PMID: 28764788
  10. The acquired sensitivity to erlotinib supports the known crosstalk between MET and the HER family of receptors. For the first time, we show inactivation of NF2 during acquisition of resistance to MET-TKI that may explain the refractoriness to erlotinib in these cells. PMID: 28396363
  11. Genetic data coupled with transcriptomic data allowed the identification of a new malignant pleural mesothelioma (MPM)molecular subgroup, C2(LN), characterized by a co-occurring mutation in the LATS2 and NF2 genes in the same MPM. MPM patients of this subgroup presented a poor prognosis. Coinactivation of LATS2 and NF2 leads to loss of cell contact inhibition between MPM cells PMID: 28003305
  12. The occurrance and evolvement of sporadic intraspinal Schwannomas have a close relationship with mutations of the NF2 gene. PMID: 28981922
  13. In this study, the authors perform an exome, methylation and RNA-seq analysis of 31 cases of radiation-induced meningioma and show NF2 rearrangement, an observation previously unreported in the sporadic tumors. PMID: 28775249
  14. An independent set of Sarcomatoid Renal Cell Carcinoma demonstrated mutations in NF2. NF2 mutations were mutually exclusive with TP53 but not with VHL mutations. PMID: 26895810
  15. sustained activation of Wnt/beta-catenin signaling due to abrogation of Merlin-mediated inhibition of LRP6 phosphorylation may be a cause of Neurofibromatosis type II disease. PMID: 27285107
  16. Study demonstrated a high frequency of structural variants, including novel truncating fusions of NF2, and an HRR-independent evolution of AC3 signature in low-dose radiation-induced meningiomas. PMID: 28474103
  17. Four of the five had a mutation in the NF2 gene. Three had a family history of NF2; one of these patients also had a family history of intracranial aneurysm with NF2 PMID: 28429859
  18. Molecular analyses for NF2 mutations in blood of the irradiated individuals failed to detect disease-causing mutations PMID: 28422417
  19. We suggest that PrP(C) and its interactor, LR/37/67 kDa, could be potential therapeutic targets for schwannomas and other Merlin-deficient tumours. PMID: 28692055
  20. These findings uncover the significance of Merlin protein expression and Survivin labeling index as prognosticators for poor clinical outcome in two independent Malignant pleural mesothelioma cohorts. PMID: 27378628
  21. NF2 localizes in nucleus when Ser518 is not phosphorylated, while phosphorylated form is present in cytoplasm and plasma membrane. Data suggest that binding of NF2 to TIMAP and EBP50 is critical in nuclear localization of NF2. (NF2 = neurofibromin 2; TIMAP = TGF-beta-inhibited membrane-associated protein; EBP50 = Ezrin-Radixin-Mosein binding phosphoprotein 50) PMID: 27871951
  22. Low merlin expression is associated with meningioma and schwannoma. PMID: 28729415
  23. Collectively, we provide for the first time in vivo evidence that the function of Merlin, as a tumor suppressor is independent of its conformational change. PMID: 28919412
  24. Data show that neurofibromin 2 (Merlin) suppresses proliferation and adhesion, at least partly, through inhibiting kinase suppressor of Ras 1 (KSR1) and DCAF1 protein. PMID: 26549023
  25. IL-1beta Induces NF2 Promoter Methylation in Meningioma/Leptomeningeal Cells. PMID: 26840621
  26. The authors proposed that NF2 behaves as a protein sensing tissue damage and aromatase-driven local estrogen formation, eventually leading to regulation of stem cells differentiation and tissue repair by liver cancer cells. (Review) PMID: 27289045
  27. co-deletion of Rac1 with Nf2 blocks tumor initiation but paradoxically exacerbates hepatomegaly induced by Nf2 loss, which can be suppressed either by treatment with pro-oxidants or by co-deletion of Yap. PMID: 27818180
  28. Data suggest that, at least using the commercial antibodies used in this study, immunohistochemical staining for NF2 (neurofibromin 2), LATS2 (large tumor suppressor kinase 2), and YAP/TAZ (nuclear translocation of complex of Yes-associated protein [YAP] with transcriptional coactivator with PDZ-binding motif [TAZ]) is not helpful for differential diagnosis of mesothelioma versus a benign proliferation. PMID: 27128293
  29. The mortality of patients with NF2 diagnosed in more recent decades was lower than that of patients diagnosed earlier. PMID: 26275417
  30. AMOTL1 Promotes Breast Cancer Progression and Is Antagonized by Merlin PMID: 26806348
  31. homozygous deletions in CDKN2A and hemizygous loss of NF2 as detected by fluorescence in situ hybridization would confer a poor clinical outcome and may guide future treatment decisions for patients with peritoneal mesothelioma. PMID: 26493618
  32. NF2/merlin inactivation augments mutant RAS signaling by promoting YAP/TEAD-driven transcription of oncogenic and wild-type RAS, resulting in greater MAPK output and increased sensitivity to MEK inhibitors. PMID: 26359368
  33. Loss of Nf2 and Cdkn2a/b have synergistic effects with PDGF-B overexpression promoting meningioma malignant transformation. PMID: 26418719
  34. we demonstrate that NF2 negatively controls the invasiveness of Glioblastoma multiforme through YAP-dependent induction of CYR61/CCN1 and miR-296-3p. PMID: 26923924
  35. angiomotin and Merlin respectively interface cortical actin filaments and core kinases in Hippo signaling PMID: 26045165
  36. (Delta2-4)Merlin variant disrupts the normal function of Merlin and promotes hepatocellular carcinoma metastasis. PMID: 26443326
  37. Stusies indicate that monosomy 22, which is often associated with mutations of the neurofibromin 2 (NF2) gene, has emerged as the most frequent alteration of meningiomas. PMID: 25965831
  38. Together our results uncover miRNAs as yet another negative mechanism controlling Merlin tumor suppressor functions. PMID: 26549232
  39. NF2 (frequently deleted in MPM) inhibited Snail-mediated p53 suppression and was stabilized by RKIP. PMID: 25823924
  40. findings demonstrated that Merlin critically regulated pancreatic cancer pathogenesis by suppressing FOXM1/beta-catenin signaling PMID: 26483206
  41. Mutation in NF2 gene is associated with malignant peritoneal mesothelioma. PMID: 25798586
  42. The p53/mouse double minute 2 homolog complex deregulation in merlin-deficient tumours. PMID: 25217104
  43. Findings suggest that the majority of NF2-associated vestibular schwannomas are polyclonal, such that the tumor mass represents a collision of multiple, distinct tumor clones. PMID: 25452392
  44. identified potential driver mutations in NF2 (neurofibromatosis type 2) and MN1 (meningioma 1). PMID: 25549701
  45. Our findings demonstrated that merlin exerts inhibitory effects on TNF-alpha-induced EMT by regulating hyaluronan endocytosis and the TAK1-p38MAPK signaling pathway. PMID: 25783601
  46. the phosphorylation of S518-Merlin in glioblastoma promotes oncogenic properties that are not only the result of inactivation of the tumor suppressor role of Merlin. PMID: 25043298
  47. These results suggest a novel tumor suppressor function of merlin in melanoma cells: the inhibition of the proto-oncogenic NHE1 activity, possibly including its downstream signaling pathways. PMID: 25275700
  48. Structural variants unique to the malignant cell line inactivated: the neurofibromin2 (NF2) gene, a known tumor suppressor. PMID: 23792589
  49. Merlin coordinates collective migration of epithelial cells by acting as a mechanochemical transducer. PMID: 25706233
  50. Integrative analysis of mutations and somatic copy-number alterations revealed frequent genetic alterations in BAP1, NF2, CDKN2A, and CUL1 in pleural mesothelioma. PMID: 25488749
  51. results led us to suggest that high frequency of NF2 mutations may play a critical role in early tumorigenesis of young vestibular schwannomas PMID: 24619252
  52. Study shows that overexpression of wild-type merlin inhibits cell proliferation, migration and adhesion. PMID: 24728215
  53. Findings indicate that merlin is sumoylated and that this post-translational modification is essential for tumor suppression. PMID: 24166499
  54. Data indicate that neurofibromin 2 (NF2) somatic mutations in 34.5% of sporadic vestibular schwannomas, but no significant difference in the mutation detection rates between cystic vestibular schwannoma and vestibular schwannoma. PMID: 24030433
  55. Data indicate that DCAF1 protein folds into a beta-hairpin structure and binds to the F3 lobe of neurofibromin 2 (Merlin) FERM domain. PMID: 24706749
  56. The NF2 L64P allele is temperature sensitive. PMID: 24595234
  57. Suggest that merlin deficiency predicts mesothelioma sensitivity to treatment with FAK inhibitors. PMID: 24848258
  58. Data indicate that neurofibromin 2 (NF2)/Merlin may serve as a potential target in the management of colorectal cancer. PMID: 24323642
  59. A deletion causing NF2 exon 9 skipping is associated with familial autosomal dominant intramedullary ependymoma. PMID: 24357459
  60. NF2/Merlin tumor suppressor function PMID: 24726726
  61. NF2 loss-driven derepressed CRL4(DCAF1) promotes activation of YAP by inhibiting hippo pathwat kinases Lats1 and 2 in the nucleus. PMID: 25026211
  62. CD43 promotes cells transformation by preventing merlin-mediated contact inhibition of growth. PMID: 24260485
  63. integrin-linked kinase (ILK) plays a critical role in the suppression of the Hippo pathway via phospho-inhibition of MYPT1-PP1, leading to inactivation of Merlin PMID: 24356468
  64. characterization of NF2 shows it binds to and stabilizes microtubules through attenuation of tubulin turnover by lowering both rates of microtubule polymerization and depolymerization as well as by reducing the frequency of microtubule catastrophes. PMID: 24282279
  65. Axonal merlin directly regulates Schwann cell behavior in patients with neurofibromatosis type 2. PMID: 24309211
  66. A clear association was found among sporadic meningiomas cases between mutation of the NF2 gene and monosomy 22. PMID: 24171707
  67. NF2 mutations may play a role in the pathophysiology of hearing loss as well as in the pattern of growth of vestibular schwannomas PMID: 23921927
  68. Intracranial meningiomas are common in NF2. They are associated with poor prognosis factors PMID: 23558725
  69. This reviews genetic properties of NF2 gene, molecular characteristics of merlin, summarizes mutational spectra and explains merlin's multifunctional roles regarding its involvement in neurofibromatosis associated tumorigenesis. PMID: 23666797
  70. Use of a common set of endpoints should improve the quality of NF2 clinical trials and will foster comparison among studies for hearing loss and facial weakness. PMID: 24249803
  71. Data indicate that the truncating mutations in NF2 protein are associated with more severe phenotypes in neurofibromatosis 2 (NF2). PMID: 22989157
  72. this is the first NF2 case caused by a deep intronic mutation in which an in vitro antisense therapeutic approximation has been tested. These results open the possibility of using this approach in vivo for this type of mutation causing NF2. PMID: 23188051
  73. Ezrin and moesin are required for efficient T cell adhesion and homing to lymphoid organs. PMID: 23468835
  74. There is a novel role for NF2 as a suppressor of JC virus T-antigen-induced cell cycle regulation. PMID: 23308224
  75. A merlin-isoform 2-dependent complex is identified that promotes neurofilament heavy chain phosphorylation. PMID: 23455610
  76. our data suggest loss of merlin results in the Rac-dependent decrease of anterograde trafficking of exocytic vesicles, representing a possible mechanism controlling the concentration of growth factor receptors at the cell surface. PMID: 22525268
  77. Merlin negatively regulates human melanoma growth, and that loss of merlin, or impaired merlin function, results in an opposite effect. PMID: 22912849
  78. Loss of the SOX10 protein, which is vital for normal Schwann cell development, is also key to the pathology of Merlin-null schwannoma tumors. PMID: 23413263
  79. LOH of the NF2 gene was observed in an early stage of WDPMP, thus indicating that LOH of the NF2 gene is an early molecular alteration, and NF2 loss is a molecular mechanism associated not only with malignant pleural mesothelioma, but also with WDPMP. PMID: 23036697
  80. The frequency of NF2 allelic losses seen in Croatian patients is broadly similar to that reported in other populations and thus confirms the existing hypothesis regarding the tumorigenesis of schwannomas, and contributes to schwannoma genetic profile. PMID: 22911524
  81. Merlin's role in restricting cortical Ezrin may contribute to tumorigenesis by disrupting cell polarity, spindle orientation, and, potentially, genome stability PMID: 23249734
  82. The C-terminal domains are important for merlin's morphogenic properties and growth inhibiting function. PMID: 22325036
  83. Merlin protein might contribute to the initiation of metastasis of non-small cell lung carcinoma. PMID: 21743150
  84. NF2 gene inactivation by promoter hypermethylation is a rare or very uncommon mechanism of NF2 gene inactivation in sporadic VS. PMID: 22249120
  85. alternative splicing and frequent codeletion of CHEK2 and NF2 contribute to the genomic instability and associated development of aggressive biologic behavior in meningiomas. PMID: 22355270
  86. The molecular genetic changes in sporadic VS identified here included mutations and allelic loss, but no aberrant hypermethylation of the NF2 gene was detected PMID: 22295085
  87. We report the first description of a pigmented choroidal schwannoma in PTEN hamartoma tumor syndrome (PHTS). This rare tumor showed a unique combination of reduction of PTEN and absence of NF2 expression. PMID: 22281088
  88. The mechanism by which merlin loss leads to increased Wnt/beta-catenin in NF2 was investigated. PMID: 22247700
  89. in addition to the tumor-suppressing activity of merlin, it also functions to maintain physiological angiogenesis in the nervous system by regulating antiangiogenic factors such as SEMA3F PMID: 22431917
  90. Our data indicate that somatic mutation of NF2 gene is not prevalent in common human cancers, and its mutation somatically occurs in a minor fraction of HCC, lung cancer and acute leukaemia. PMID: 22081132
  91. gastrointestinal stromal tumor cases exhibiting expression of phosphorylated Thr567 in the ezrin protein were associated with immunoactivities of KIT and merlin expression PMID: 21971708
  92. The study reports the crystal structure of the human merlin head domain when crystallized in the presence of its tail domain. PMID: 22012890
  93. a novel mechanism for the loss of Merlin protein in breast cancer, and have developed a discriminatory model using Merlin and OPN expression in breast tumor tissues. PMID: 21965655
  94. Sequential phosphorylation of merlin C- and N-terminus by different oncogenic kinases targets merlin for degradation and thus downregulates its activity. PMID: 21750658
  95. The effects of splicing mutations in NF2 are often complex and that information theory based analysis is helpful in elucidating the consequences of these mutations. PMID: 21563229
  96. Results demonstrate that Schwannomin plays an essential role in inducing and/or maintaining the SC's spindle shape, and by stabilizing the bipolar morphology, Sch promotes the alignment of SCs with axons and ultimately influences myelin segment length. PMID: 21182951
  97. Mutations within the NF2 gene in Neurofibromatosis type 2 patients are associated with the risk of developing meningiomas PMID: 21278391
  98. Depletion of Angiomotin in Nf2(-/-) Schwann cells attenuates the Ras-MAPK signaling pathway, impedes cellular proliferation in vitro and tumorigenesis in vivo PMID: 21481793
  99. merlin mediates PICT-1-induced growth inhibition by translocating to the nucleolus and binding PICT-1 PMID: 21167305
  100. A four-hit mechanism of tumour suppressor gene inactivation, involving SMARCB1 and NF2, might be operative in familial multiple meningiomas associated meningiomas. PMID: 20930055

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Involvement in disease Neurofibromatosis 2 (NF2); Schwannomatosis 1 (SWNTS1); Mesothelioma, malignant (MESOM)
Subcellular Location Isoform 1: Cell projection, filopodium membrane, Peripheral membrane protein, Cytoplasmic side, Cell projection, ruffle membrane, Peripheral membrane protein, Cytoplasmic side, Nucleus, Note=In a fibroblastic cell line, isoform 1 is found homogeneously distributed over the entire cell, with a particularly strong staining in ruffling membranes and filopodia, Colocalizes with MPP1 in non-myelin-forming Schwann cells, Binds with DCAF1 in the nucleus, The intramolecular association of the FERM domain with the C-terminal tail promotes nuclear accumulation, The unphosphorylated form accumulates predominantly in the nucleus while the phosphorylated form is largely confined to the non-nuclear fractions, SUBCELLULAR LOCATION: Isoform 7: Cytoplasm, perinuclear region, Cytoplasmic granule, Note=Observed in cytoplasmic granules concentrated in a perinuclear location, Isoform 7 is absent from ruffling membranes and filopodia, SUBCELLULAR LOCATION: Isoform 9: Cytoplasm, perinuclear region, Cytoplasmic granule, Note=Observed in cytoplasmic granules concentrated in a perinuclear location, Isoform 9 is absent from ruffling membranes and filopodia, SUBCELLULAR LOCATION: Isoform 10: Nucleus, Cell projection, filopodium membrane, Peripheral membrane protein, Cytoplasmic side, Cell projection, ruffle membrane, Peripheral membrane protein, Cytoplasmic side, Cytoplasm, perinuclear region, Cytoplasmic granule, Cytoplasm, cytoskeleton
Tissue Specificity Widely expressed. Isoform 1 and isoform 3 are predominant. Isoform 4, isoform 5 and isoform 6 are expressed moderately. Isoform 8 is found at low frequency. Isoform 7, isoform 9 and isoform 10 are not expressed in adult tissues, with the exception of adul
Database Links

HGNC: 7773

OMIM: 101000

KEGG: hsa:4771

STRING: 9606.ENSP00000344666

UniGene: Hs.187898

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