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  4. Recombinant Human Thrombopoietin receptor (MPL), partial

Recombinant Human Thrombopoietin receptor (MPL), partial

In Stock
  • 货号：
    CSB-EP014755HU
  • 规格：
    ￥1344
  • 图片：
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他：

产品详情

  • 纯度：
    Greater than 90% as determined by SDS-PAGE.
  • 基因名：
  • Uniprot No.：
  • 别名：
    C MPL; CD110; mpl; MPLV; Myeloproliferative leukemia protein; Myeloproliferative leukemia virus oncogene; Proto-oncogene c-Mpl; THCYT2; Thrombopoietin receptor; TPO R; TPO-R; TPOR; TPOR_HUMAN
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Extracellular Domain
  • 来源：
    E.coli
  • 分子量：
    56.5kDa
  • 表达区域：
    26-491aa
  • 氨基酸序列
    QDVSLLASDSEPLKCFSRTFEDLTCFWDEEEAAPSGTYQLLYAYPREKPRACPLSSQSMPHFGTRYVCQFPDQEEVRLFFPLHLWVKNVFLNQTRTQRVLFVDSVGLPAPPSIIKAMGGSQPGELQISWEEPAPEISDFLRYELRYGPRDPKNSTGPTVIQLIATETCCPALQRPHSASALDQSPCAQPTMPWQDGPKQTSPSREASALTAEGGSCLISGLQPGNSYWLQLRSEPDGISLGGSWGSWSLPVTVDLPGDAVALGLQCFTLDLKNVTCQWQQQDHASSQGFFYHSRARCCPRDRYPIWENCEEEEKTNPGLQTPQFSRCHFKSRNDSIIHILVEVTTAPGTVHSYLGSPFWIHQAVRLPTPNLHWREISSGHLELEWQHPSSWAAQETCYQLRYTGEGHQDWKVLEPPLGARGGTLELRPRSRYRLQLRARLNGPTYQGPWSSWSDPTRVETATETAW
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal 6xHis-tagged
  • 产品提供形式：
    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.
  • 缓冲液：
    Tris-based buffer,50% glycerol
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    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.
  • 货期：
    3-7 business days
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • 产品描述：

    The Recombinant Human MPL protein is a protein encoded by recombinant DNA that was cloned in an expression vector that supported the expression of MPL gene. This recombinant MPL protein was expressed in the host. The expression region is 26-491aa of the Human MPL. In the production, the expression vector contains N-terminal 6xHis tag. Every production step was performed with a strict QC system. The purity of this protein is 90%+ determined by SDS-PAGE.

    The thrombopoietin receptor, MPL, is a member of the type 1 homodimeric cytokine receptor family together with the EPO, prolactin, growth hormone, and granulocyte-colony stimulating factor receptors. Among this receptor family, MPL is unique because it has a reduplicated extracellular CRHD, of which, the distal CRHD is the site of THPO binding. The distal CRHD is also the site of germline SNP causing benign familial thrombocytosis, a hot spot for the mutations causing CAMT as well as the site of terminal sialylation during receptor maturation, which is impaired in the MPN. Like the other hematopoietic growth factor receptors, MPL is responsible for the metabolism of its cognate ligand, but in contrast to the other hematopoietic growth factors, the production of which is mainly regulated by demand. MPL offers a selective therapeutic target in the myeloproliferative neoplasms since impaired MPL processing is unique to the involved stem cells, while MPL is required for hematopoietic stem cell survival and quiescent in their bone marrow niches.

  • Datasheet & COA：
    Please contact us to get it.

产品评价

靶点详情

  • 功能：
    Receptor for thrombopoietin that acts as a primary regulator of megakaryopoiesis and platelet production. May represent a regulatory molecule specific for TPO-R-dependent immune responses.
  • 基因功能参考文献：
    1. JAK2V617F mutation was found in 37 (61.7%) patients with ET. Among 23 patients without JAK2V617F mutation, 7 (11.7%) had CALR mutation and 1 (1.7%) had MPL mutation. Fifteen (25.0%) patients were negative for all 3 mutations: JAK2V617F(-), CALR(-), and MPL(-). PMID: 29390868
    2. MPL and CALR genotypes show a similar clinical picture at essential thrombocythaemia diagnosis. Bone marrow histology in MPL-mutated ET is characterized by prominent megakaryocytic proliferation. PMID: 29934356
    3. These results indicate that lusutrombopag acts on human TPOR to upregulate differentiation and proliferation of megakaryocytic cells, leading to platelet production. PMID: 29274361
    4. The expression of TPO and c-Mpl was significantly decreased in the cITP group compared to the nITP group, suggesting that TPO and its receptor may play important roles in childhood cITP pathogenesis. PMID: 29313460
    5. A novel germ-line mutation of c-mpl gene in a sporadic case of essential thrombocythemia. PMID: 28391042
    6. This study demonstrated that absence of MPL mutation in stroke. PMID: 28625126
    7. MPL mutations and splenomegaly are risk factors for essential thrombocythemia progression into primary nyelofibrosis. PMID: 27768091
    8. goals were: (i) to identify other MPL mutations that should be tested in MPN patients by mutation-specific PCR; and (ii) to determine the amino acid requirements at position 515 to prevent TpoR self-activation PMID: 26437785
    9. Concurrent MPL W515L and Y591D mutations in a patient with myelofibrosis. PMID: 27519934
    10. MPL is up regulated in JAK2(V617F) ECs and contributes to the maintenance/expansion of the JAK2(V617F) clone over JAK2(WT) clone in vitro PMID: 27865175
    11. In tumor cell cultures, exogenous expression of MPL led to constitutive activation of STAT3 and 5, ERK1/2, and AKT, cytokine-independent growth, and reduction of apoptosis similar to the effects seen in the spontaneously outgrown cells. PMID: 27177927
    12. Essential Thrombocythemia and Primary Myelofibrosis patients with MPL mutations are at high risk for Thrombotic Events. PMID: 28766534
    13. Results show that mutant CALR induces autocrine, but not paracrine activation of MPL in myeloproliferative neoplasm. [review] PMID: 28741795
    14. these results demonstrate that MPL P106L is a receptor with an incomplete defect in trafficking. PMID: 28034873
    15. A newborn girl with congenitcal amegakaryocytic thrombocytopenia had a homozygous missense Trp154-to- Arg mutation in exon 4 of c-MPL. The same heterozygote mutation was detected in her mother, father, and 2 siblings. PMID: 26316487
    16. Normal FLT3 and negative expression of CD34 and cMPL may predict a longer overall survival in aute myeloid leukemia. PMID: 27993871
    17. we show that the positive charge of the CALR mutant C-terminus is necessary to transform hematopoietic cells by enabling binding between mutant CALR and the thrombopoietin receptor MPL. PMID: 26951227
    18. In essential thrombocythemia, MPL mutations might be associated with a higher risk of fibrotic transformation and the presence of JAK2/MPL mutations with higher risk of thrombosis. PMID: 26890983
    19. PARP-1 has an important role in the progression of acute myeloid leukemia by suppressing the myeloproliferative leukemia virus oncogene PMID: 26314963
    20. mutant CALR promotes myeloproliferative neoplasm development by activating c-MPL and its downstream pathway. PMID: 26817954
    21. Thrombopoietin receptor activation by myeloproliferative neoplasm associated calreticulin mutants. PMID: 26668133
    22. we describe a Mpl W515K somatic mutation in a paediatric case of ET who presented with Budd-Chiari syndrome. No paediatric patient harbouring a Mpl W515K mutation has been previously reported. PMID: 25970554
    23. His(499) regulates the activation of human TpoR and provides additional protection against activating mutations, such as oncogenic Asn mutations in the TM domain PMID: 26627830
    24. this study has shown that in a fraction of the so-called triple-negative ETs a significant proportion of patients have mutations in signaling molecules, more particularly in MPL. PMID: 26450985
    25. erythrocyte lineage enforces exclusivity through upregulation of EKLF and its lineage-specific cytokine receptor (EpoR) while inhibiting both FLI-1 and the receptor TpoR (also known as MPL) for the opposing megakaryocyte lineage PMID: 26159733
    26. Compared to normal controls, the frequency of the JAK246/1 haplotype was significantly higher among patients with JAK2V617F, JAK2Ex12del, or MPL mutations, whereas no significant difference was found among CALR mutation-positive patients PMID: 26614694
    27. Flow cytometric detection of MPL (CD110) as a diagnostic tool for differentiation of congenital thrombocytopenias. PMID: 25911549
    28. Using C-mannosylation defective mutant of c-Mpl, the C-mannosylated tryptophan residues at four sites (Trp(269), Trp(272), Trp(474), and Trp(477)) are essential for c-Mpl-mediated JAK-STAT signaling. PMID: 26505790
    29. MPL gene mutations are associated with essential thrombocythaemia and major thrombotic complications. PMID: 25573593
    30. Letter/Meta-analysis: thrombopoietin receptor agonists significantly increase the risk of portal vein thrombosis in liver diseases. PMID: 25761530
    31. MPL mutation is associated with myeloproliferative neoplasms. PMID: 25398833
    32. The effects of inhibition of the TPO/c-MPL pathway on enhancing the chemotherapy sensitivity of AML cells. PMID: 24085601
    33. The data supports the proposal of including MPL exon 10 mutations as major diagnostic markers for myeloproliferative neoplasms. PMID: 26071474
    34. CALR mutation, MPL mutation and triple negativity may have roles in lowering vascular risk in primary myelofibrosis PMID: 25482134
    35. Both immature and mature Mpl reach the cell surface. PMID: 24931576
    36. The P106L mutation functionally separates The activity of c-Mpl in downstream signaling from that in maintaining platelet homeostasis. PMID: 25538044
    37. Amino acid substitutions in a thrombopoietin receptor (Mpl)--containing cell growth switch (CGS) extending receptor stability improve the expansion capacity of human cord blood CD34(+) cells in the absence of exogenous cytokines. PMID: 25343958
    38. OTT1 regulates the alternative splicing of Mpl-TR, a truncated isoform of c-Mpl, which modulates Thrombopoietin-mediated signaling. PMID: 25468569
    39. Studies demonstrate that progression to AML is part of the natural history of MPL W515L-associated disease. PMID: 20823136
    40. Impaired transcriptional regulation of the MPL signaling that normally governs megakaryopoiesis and erythropoiesis underlies congenital amegakaryocytic thrombocytopenia. PMID: 23908116
    41. These experiments define a novel VEGF-miR-1-Mpl-P-selectin effector pathway in lung Th2 inflammation and herald the utility of miR-1 and Mpl as potential therapeutic targets for asthma. PMID: 24043765
    42. In migrating cancer stem cells isolated from primary human colorectal cancers, CD110(+) and CDCP1(+) subpopulations mediate organ-specific lung and liver metastasis. PMID: 23747337
    43. MPL W515L mutation in pediatric essential thrombocythemia. PMID: 23441089
    44. Different mutations of the human c-mpl gene indicate distinct hematopoietic diseases. PMID: 23351976
    45. Loss of heterozygosity of chromosome 1p involving the MPL location may represent a molecular mechanism of fibrotic transformation in MPL-mutated myeloproliferative neoplasms. PMID: 23575445
    46. MPL Baltimore mutation is associated with thrombocytosis. PMID: 23511495
    47. Tryptophan at the transmembrane-cytosolic junction modulates thrombopoietin receptor dimerization and activation. PMID: 23359689
    48. Up-regulation of wild-type MPL levels promotes leukemia development and maintenance through activation of the PI3K/AKT axis. PMID: 22613795
    49. High MPL expression is associated with leukemia. PMID: 22337712
    50. This study shows for the first time a link between homozygous MPL mutations and familial aplastic anemia. It also highlights the important role of MPL in trilineage hematopoiesis. PMID: 22180433

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  • 相关疾病：
    Congenital amegakaryocytic thrombocytopenia (CAMT); Thrombocythemia 2 (THCYT2); Myelofibrosis with myeloid metaplasia (MMM)
  • 亚细胞定位：
    Cell membrane; Single-pass type I membrane protein. Golgi apparatus. Cell surface.
  • 蛋白家族：
    Type I cytokine receptor family, Type 1 subfamily
  • 组织特异性：
    Expressed at a low level in a large number of cells of hematopoietic origin. Isoform 1 and isoform 2 are always found to be coexpressed.
  • 数据库链接：

    HGNC: 7217

    OMIM: 159530

    KEGG: hsa:4352

    STRING: 9606.ENSP00000361548

    UniGene: Hs.82906