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  4. Recombinant Human Bone morphogenetic protein receptor type-2 (BMPR2), partial

Recombinant Human Bone morphogenetic protein receptor type-2 (BMPR2), partial

  • 货号：
    CSB-YP618807HU
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 货号：
    CSB-EP618807HU
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 货号：
    CSB-EP618807HU-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    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：
  • 货号：
    CSB-BP618807HU
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 货号：
    CSB-MP618807HU
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
  • Uniprot No.：
  • 别名：
    BMP type II receptor; BMP type-2 receptor; BMPR 2; BMPR 3; BMPR II; BMPR-2; BMPR-II; Bmpr2; BMPR2_HUMAN; BMPR3; BMPRII; BMR 2; BMR2; Bone morphogenetic protein receptor type 2; Bone morphogenetic protein receptor type II; Bone morphogenetic protein receptor type-2; Bone morphogenic protein receptor type II serine threonine kinase; BRK 3; BRK3; PPH 1; PPH1; Serine threonine kinase type II activin receptor like kinase; T ALK; TALK; Type II activin receptor like kinase
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Partial
  • 蛋白标签：
    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.
  • 产品提供形式：
    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.
  • 复溶：
    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.
  • 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：
    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.
  • 注意事项：
    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.

产品评价

靶点详情

  • 功能：
    On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Binds to BMP7, BMP2 and, less efficiently, BMP4. Binding is weak but enhanced by the presence of type I receptors for BMPs. Mediates induction of adipogenesis by GDF6.
  • 基因功能参考文献：
    1. Heterozygous germline mutations in the gene coding bone morphogenetic receptor type 2 (BMPR2) are detectable in the majority of cases of heritable pulmonary arterial hypertension, and in approximately 20% of cases of idiopathic pulmonary arterial hypertension. [review] PMID: 29032562
    2. Tumour necrosis factor-alpha selectively reduces BMPR-II transcription and mediates post-translational BMPR-II cleavage via the sheddases, ADAM10 and ADAM17 in pulmonary artery smooth muscle cells. PMID: 28084316
    3. miR-23a facilitated cell proliferation and migration by targeting BMPR2/Smad1 signaling in hypoxia-induced human pulmonary artery smooth muscle cells. PMID: 29864909
    4. we employed an shRNA-encoding lentivirus system to inhibit SPG6 expression in AML cells including NB4 and MV4-11cells. Knockdown expression of SPG6 resulted in decreased cell growth and elevated apoptosis of these leukemia cells. Notably, SPG6 deficiency resulted in higher BMPR2 expression indicating that BMPR2 signaling contributes to AML pathogenesis. PMID: 29715457
    5. Sequencing of BMPR2, CAV1, and KCNK3 coding regions did not identify any pathogenic variants in these genes in infants with pulmonary hypoplasia and pulmonary hypertension. PMID: 28162765
    6. The present study showed that deletion-duplication mutations in the BMPR2 or ACVRL1 genes may not be associated with non-regression of Pulmonary arterial hypertension. PMID: 28290170
    7. BMPR2 mutation carriers are more prone to hemoptysis and that hemoptysis is closely correlated to bronchial arterial remodelling and angiogenesis; in turn, pronounced changes in the systemic vasculature correlate with increased pulmonary venous remodelling, creating a distinctive profile in pulmonary arterial hypertension patients harbouring a BMPR2 mutation. PMID: 27811071
    8. Studying the methylation pattern of the BMPR2 promoter region in pulmonary arterial hypertension patients and controls revealed a a CpG island, suitable for methylation, in the BMPR2 promoter region, in addition to NIT-2, sex-determining region Y, and heat shock factor transcription factor binding sites.No evidence of methylation was detected in this region in patients and controls. PMID: 26654628
    9. Mutations in the bone morphogenetic protein receptor type-2 gene (BMPR2) have been identified in patients with pulmonary arterial hypertension. PMID: 27248591
    10. Affected mutation carriers with heritable pulmonary hypertension have hypermethylation of the BMPR2 promotor compared with their unaffected relatives. PMID: 28170297
    11. increased BMPR2 signal transduction is linked to fragile X syndrome (FXS) and that the BMPR2-LIMK1 pathway is a putative therapeutic target in patients with FXS and possibly other forms of autism PMID: 27273096
    12. A burden of rare variants in BMPR2 significantly contributed to the risk of pulmonary arterial hypertension. In the remaining one family, the patient carried a pathogenic variant in a member of potassium channels, KCNK3, which was the first replicative finding of channelopathy in an Asian population. PMID: 28388887
    13. The SMYD2 may promote BMP signaling by directly methylating BMPR2, which, in turn, stimulates BMPR2 kinase activity and activation of the BMP pathway. PMID: 28588028
    14. This review focuses on recent advances in rescuing BMPRII expression, function or signaling to prevent and reverse pulmonary vascular remodeling in pulmonary arterial hypertension and its feasibility for clinical translation. Furthermore, it summarizes the role of described miRNAs that directly target the BMPR2 gene in blood vessels. [review] PMID: 28447104
    15. Endothelial BMPR2 signaling in pulmonary arterial hypertension is impaired by deletion of Vegfr3. PMID: 28356442
    16. Disrupting BMPR2 impairs TGFbeta1- and BMP4-mediated elastic fiber assembly and is of pathophysiologic significance in pulmonary arterial hypertension. PMID: 28619995
    17. Cav-1 depletion, oxidative stress-mediated reduction in BMPRII expression, and enhanced TGF-beta-driven SMAD-2/3 signaling promote pulmonary vascular remodeling in inflamed lungs. PMID: 28188225
    18. This analysis identified features of unaffected mutation carriers iPSC-induced pluripotent stem cell-derived endothelial cells related to modifiers of BMPR2 signaling or to differentially expressed genes. PMID: 28017794
    19. Decreased expression of bone morphogenetic protein receptor type 2 (BMPR2) is associated with all forms of PAH, and a mutation in this receptor is seen in 70% of patients with the heritable form of PAH (HPAH), and in 20% of sporadic cases of idiopathic PAH. PMID: 27779452
    20. HPAH-associated BMPRII mutation increases pulmonary microvascular endothelial cells adhesiveness for monocytes in response to inflammatory mediators. PMID: 27816994
    21. BMPR2 downregulation may have a role in neuroblastoma PMID: 27998774
    22. Bone morphogenetic protein 2 expression increases and may contribute to partitioning of energy storage into visceral and subcutaneous AT depots PMID: 27515773
    23. Depletion of BMPR2 mediated by a collection of miRs induced by IL6 and subsequent STAT3 phosphorylation is a novel mechanism participating to fibroproliferative and vascular injuries in idiopathic pulmonary fibrosis. PMID: 27317687
    24. Pathogenic BMPR2 mutations were identified in 8 of 72 (11.1%) patients with IPAH and 6 of 9 (66.7%) patients with HPAH. PMID: 27884767
    25. In a cohort with idiopathic or hereditary pulmonary arterial hypertension, a possibly associated mutation was found in 11.10% of the idiopathic cases (n = 16) and in 68.18% of the hereditary cases. There were 19 mutations found in BMPR2. PMID: 27453251
    26. Case Report: sarcoid-like reaction due to pulmonary hypertension in the context of the BMPR2 mutation. PMID: 27537724
    27. Patients with pulmonary arterial hypertension and bone morphogenetic protein receptor type II mutations present at a younger age with more severe disease, and are at increased risk of death, and death or transplantation, compared with those without BMPR2 mutations. PMID: 26795434
    28. Study of four patients with pulmonary arterial hypertension associated with human immunodeficiency virus infection found predisposing mutations in the BMPR2, ACVRL1 and ENG genes. PMID: 26897508
    29. BMPR2 mutations were identified in congenital heart disease-pulmonary vascular disease patients, with missense mutation of BMPR2 as the dominant mutation type. PMID: 27002414
    30. his study demonstrated that both rs6435156C > T and rs1048829G > T variants in BMPR2 contributed to increased susceptibility to hronic obstructive pulmonary disease PMID: 27077124
    31. Increased HMGA1 in pulmonary arterial endothelial cells resulting from dysfunctional BMPR2 signaling can transition endothelium to smooth muscle-like cells associated with pulmonary arterial hypertension. PMID: 27045138
    32. Pulmonary arterial hypertension patients carrying a BMPR2 mutation have decreased right ventricular function compared to patients without the mutation. PMID: 26984938
    33. the data in the present study support the notion that the expression levels and plasma membrane levels of BMPRII are determined by two molecular processes-translational regulation of protein synthesis (which provides the major contribution) and endocytosis/degradation (mild modulatory effect). PMID: 26739752
    34. In a group of pulmonary arterial hypertension patients, 25.4% harboured heterozygous mutation in the BMPR2 gene. PMID: 26541523
    35. establish the feasibility of combining NELL-1 with BMP2 to improve clinical bone regeneration and provide mechanistic insight into canonical Wnt pathway activity during NELL-1 and BMP2 osteogenesis PMID: 26772960
    36. Raf family members and ERK1/2 were constitutively activated after BMPR2 knockdown PMID: 26589479
    37. miR-153 is a mechano-sensitive miRNA that regulates osteoblast differentiation by directly targeting BMPR2, and that therapeutic inhibition of miR-153 may be an efficient anabolic strategy for skeletal disorders caused by pathological mechanical loading. PMID: 26151470
    38. study shows for the first time that in the regulatory region of the BMPR2 gene the promoter may be important for pulmonary arterial hypertension penetrance PMID: 26167679
    39. Correlations between C23, BMPRII expression and prognosis of gastric cancer patients. PMID: 25698539
    40. local gene transfection can up-regulate the expression of osteogenic mediators (BMP-2 and TGF-beta1), which may promote cell differentiation and proliferation and stimulate extracellular matrix synthesis and new bone formation in distraction gap. PMID: 25723654
    41. BMP2 decreased serum-induced proliferation and increased the pro-apoptotic Bax/Bcl-2 ratio. These effects were attenuated by endothelin-1 pre-treatment PMID: 25447587
    42. our results showed that GDF-5 and BMPRII expressed both in normal and degenerated intervertebral disc tissues, and GDF-5 might have an inhibition effect on degenerated lumbar intervertebral discs PMID: 25755766
    43. The BMPR2 protein containing Thr268fs, Ser863Asn, or Gln433X exhibited abnormal subcellular localization. PMID: 25187962
    44. Mutations in BMPR2 gene is associated with pulmonary arterial hypertension. PMID: 24936649
    45. Mutations in BMPR2 underlie most heritable cases and a small proportion of sporadic cases of idiopathic pulmonary arterial hypertension. Read More: http://www.atsjournals.org/doi/full/10.1164/rccm.201408-1528OC#.Viqgi9KFPyA PMID: 26030479
    46. BMP9 is identified as the preferred ligand for preventing apoptosis and enhancing monolayer integrity in endothelial cells from subjects with pulmonary arterial hypertension who bear mutations in the gene encoding BMPR2. PMID: 26076038
    47. Silencing BMPR2 promoted G2/M cell cycle arrest and apoptosis through caspase-3-dependent pathway via repression of XIAP and induced autophagy of chondrosarcoma cells via XIAP-Mdm2-p53 pathway. PMID: 25501832
    48. Mutations in BMPR2 encoding bone morphogenetic protein receptor type 2 (BMPRII) is the main genetic risk factor for heritable pulmonary arterial hypertension PMID: 25429696
    49. disrupted intracellular trafficking of BMPR2 is involved in the pathogenic mechanism underlying both cysteine and non-cysteine substitutions occurring in the extracellular ligand binding domain and kinase domain of BMPR2. PMID: 25688877
    50. Combining mutation detection in family members with parental identification, study described three cases of de novo mutation in the BMPR2 gene by different modes in a pulmonary arterial hypertension family. These de novo mutations may account for the wide variety of mutations in BMPR2. PMID: 25612240

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  • 相关疾病：
    Pulmonary hypertension, primary, 1 (PPH1); Pulmonary venoocclusive disease 1, autosomal dominant (PVOD1)
  • 亚细胞定位：
    Cell membrane; Single-pass type I membrane protein.
  • 蛋白家族：
    Protein kinase superfamily, TKL Ser/Thr protein kinase family, TGFB receptor subfamily
  • 组织特异性：
    Highly expressed in heart and liver.
  • 数据库链接：

    HGNC: 1078

    OMIM: 178600

    KEGG: hsa:659

    STRING: 9606.ENSP00000363708

    UniGene: Hs.471119