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  4. SDHB Recombinant Monoclonal Antibody

SDHB Recombinant Monoclonal Antibody

  • 货号：
    CSB-RA987662A0HU
  • 规格：
    ￥1320
  • 图片：
    • Western Blot
      Positive WB detected in: 293 whole cell lysate, K562 whole cell lysate, NIH/3T3 whole cell lysate, PC-3 whole cell lysate, Rat heart tissue, Mouse heart tissue, Mouse liver tissue
      All lanes: SDHB antibody at 1:2000
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 32 kDa
      Observed band size: 32 kDa
    • IHC image of CSB-RA987662A0HU diluted at 1:100 and staining in paraffin-embedded human heart tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
    • IHC image of CSB-RA987662A0HU diluted at 1:100 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
    • Overlay histogram showing 293 cells stained with CSB-RA987662A0HU (red line) at 1:50. The cells were fixed with 70% Ethylalcohol (18h) and then incubated in 10% normal goat serum to block non-specific protein-protein interactions followedby the antibody (1µg/1*106 cells) for 1 h at 4℃.The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 30min at 4℃. Control antibody (green line) was Rabbit IgG (1µg/1*106 cells) used under the same conditions. Acquisition of >10,000 events was performed.
  • 其他：

产品详情

  • 产品描述：

    SDHB is one of the subunits of mitochondrial respiratory chain complex II. Low expression of SDHB promotes aerobic glycolysis, and SDHB function loss results in the occurrence and development of many types of tumors, including liver cancer and colorectal cancer. Lack of SDHB function is strongly linked to metabolic alterations in kidney cancer cells. Head and neck paraganglioma and phaeochromocytoma are caused by germline mutations in the SDHB gene.

    The SDHB antibody genes were cloned from B cells that were derived from immunized animals with A synthesized peptide derived from human SDHB and then introduced into the plasma vectors, which were transfected into mammalian cell lines for up-scaling expression. The product was purified by A synthesized peptide derived from human SDHB to obtain the recombinant antibody against SDHB. This recombinant SDHB antibody is reactive with the SDHB protein from Human, Mouse, Rat. It is recommended for use in the ELISA, WB, IHC, FC.

  • Uniprot No.：
    P21912
  • 基因名：
  • 别名：
    Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial (EC 1.3.5.1) (Iron-sulfur subunit of complex II) (Ip), SDHB, SDH SDH1
  • 反应种属：
    Human, Mouse, Rat
  • 免疫原：
    A synthesized peptide derived from human SDHB
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Monoclonal
  • 抗体亚型：
    Rabbit IgG
  • 纯化方式：
    Affinity-chromatography
  • 克隆号：
    7D12
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC, FC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:50-1:200
    FC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
  • 基因功能参考文献：
    1. Mutation is detected in the SDHB gene both mother and a child PMID: 30178964
    2. newly identified IVS2-2A>C mutation in SDHB is responsible for Hereditary Pheochromocytoma/Paraganglioma Syndrome. PMID: 29925701
    3. Pheochromocytoma patients with SDHB mutation carriers are at high risk for developing metastatic disease, but may respond better to CVD protocol than non-mutation carriers. If these results are confirmed in larger prospective cohorts, this could affect choice and possibly timing of chemotherapy in SDHB patients with consideration to giving CVD earlier in the treatment plan to SDHB mutation carriers. PMID: 28566531
    4. The overall penetrance of succinate dehydrogenase B (SDHB) mutations is estimated to be 21% at age 50 and 42% at age 70 when adequately corrected for ascertainment. PMID: 28503760
    5. Germline SDHB mutation is associated with Pheochromocytoma and Paraganglioma. PMID: 28884434
    6. Data suggest that low expression of SDHB in metastatic lesions is associated with longer overall survival in patients with advanced ileal well-differentiated neuroendocrine tumors with lymph node or liver metastases. PMID: 27905048
    7. Results identified a high frequency of germline mutation in SDHB gene in patients presenting with bladder paraganglioma. PMID: 27542510
    8. automation, reproducibility, and cost efficiency of SDHB IHC offer advantages over the labor-intensive histochemical method requiring frozen sections PMID: 27556822
    9. We report a unique case of an SDH-deficient GIST case with an activating PDGFRA mutation. Oncogenic mutations in GIST are generally mutually exclusive; however documented exceptions exist which may have diagnostic and therapeutic implications. PMID: 28768491
    10. Mutation in the SDHB gene is associated with mediastinal paraganglioma. PMID: 28891197
    11. We report for the first time the presence of both TFE3 translocation and SDHB mutation in the same renal cell carcinoma tumor. PMID: 27910947
    12. Heterozygous germ line mutations in SDHB neutrophil survival is independent of HIF-1alpha expression and linked to uncoupling of the mitochondrial electron transport chain. PMID: 27006389
    13. miR-142-5p up-regulation in colorectal cancer probably facilitates generation of aerobic glycolysis by reducing SDHB expression. PMID: 28622713
    14. SDHB mutation is associated with metastatic pheochromocytoma. PMID: 28409892
    15. we found a higher estimated penetrance compared to several other studies, and a striking difference in age-related penetrance between male and female SDHB mutation carriers with no association between mutation and gender or tumor location PMID: 28374168
    16. LASS5 interacts with SDHB and synergistically represses p53 and p21 activity. PMID: 27280497
    17. A novel SDHB germline mutation in a paraganglioma PMID: 26833045
    18. SDHB-related tumours are picked up as early as 2 years after initial negative surveillance scan PMID: 27678251
    19. miR-183 cluster (miR-96/182/183) are related to clinical parameters and SDHB expression in pheochromocytomas. PMID: 28412207
    20. In SDHB mutation carriers, a lower rate of metastatic disease and a higher number of head and neck paragangliomas compared to previous reports was observed in a nationwide study in the Netherlands. PMID: 28490599
    21. Loss of FH immunohistochemical (IHC) expression in cutaneous leiomyomas is a sensitive and specific marker for detection of hereditary leiomyomatosis and renal cell carcinoma (HLRCC). FH expression by IHC was absent in 9 specimens and retained in 85 specimens and 2 cases were equivocal with minimal FH expression while succinate dehydrogenase B expression was retained in 95 specimens and equivocal in 1 specimen. PMID: 28288038
    22. Identify small molecule binding to the succinate dehydrogenase subunit B (SDHB) protein of complex II of the mitochondrial electron transfer chain to block mitochondrial apoptotic pathway. PMID: 27447985
    23. We have shown that ASO treatment diminished aberrant splicing and increased ISCU protein levels in both patient fibroblasts and patient myotubes in a concentration dependent fashion. Upon ASO treatment, levels of SDHB in patient myotubular cell lines increased to levels observed in control myotubular cell lines PMID: 28007899
    24. SDHB mutation status and primary tumor size are predictive in patient outcome diagnosed with pheochromocytoma and abdominal paraganglioma. PMID: 27839933
    25. Mutations in some enzymes of the TCA cycle: Isocitrate dehydrogenase (IDH), succinate dehydrogenase (SDH) and fumarate hydratase (FH) are associated with the accumulation of metabolites that are able to influence many aspects of cancer development and progression and for this reason are termed onco-metabolites. PMID: 27528759
    26. Heterozygous germline deletions of up to 104 kb in size were identified in SDHB, SDHC, SDHD and flanking genes in 20 paraganglioma-pheochromocytoma patients. The exact breakpoint could be determined in 16 paraganglioma-pheochromocytoma patients of which 15 were novel deletions PMID: 27485256
    27. compound germline heterozygosity of mouse Sdhb/Sdhc/Sdhd null alleles blunts chronic hypoxia-induced increases in hemoglobin levels, an adaptive response mainly regulated by HIF-2a PMID: 28204537
    28. Of which 4 SDHB and 2 TMEM127 mutations were novel. PMID: 26960314
    29. Our study, suggests that SDH loss was suggestive of metastatic behavior in addition to younger age at diagnosis, larger tumor size, and higher Ki67 proliferation rate and catecholamine type in paragangliomas of the urinary bladder PMID: 27262318
    30. Our results demonstrated that down-regulation of SDHB and up-regulation of PDK1 may be novel biomarkers for predicting advanced tumor progression and unfavorable prognosis in recurrent nasopharyngeal carcinoma patients PMID: 26547584
    31. that SDHAF1 contributes to iron-sulfur (Fe-S) cluster incorporation into the Fe-S subunit of CII, SDHB. PMID: 26749241
    32. The low penetrance of SDHB mutations may obscure the hereditary nature of SDHB-linked disease and is important in the counseling of SDHB-linked patients. PMID: 25827221
    33. Succinate dehydrogenase deficiency is a rare leukoencephalopathy, for which improved recognition by magnetic resonance imaging (MRI) in combination with advanced sequencing technologies allows noninvasive diagnostic confirmation. PMID: 26642834
    34. 15 YEARS OF PARAGANGLIOMA: Genetics and mechanism of pheochromocytoma-paraganglioma syndromes characterized by germline SDHB and SDHD mutations PMID: 26113606
    35. Study reveals new insights into the effects of SDHB mutations and the power of structural modelling in predicting biological consequences. PMID: 25972245
    36. for the first time, we show a high correlation between urinary bladder paragangliomas and SDHB mutations PMID: 25683602
    37. Findings provide a conceptual framework for understanding how particular mutations disproportionately cause the loss of SDH activity, resulting in accumulation of succinate and metabolic remodeling in SDHB cancer syndromes. PMID: 26719882
    38. Germline mutation in the SDHB gene is the only reliable predictor of malignant Paragangliomas. PMID: 24973967
    39. Report direct correlation between the presence of an SDHB mutation, whether germline or somatic, and negative SDHB immunohistochemical staining in bladder paragangliomas. PMID: 26457353
    40. Both germline and somatic SDHx mutations/variants occur in sporadic differentiated thyroid cancer (DTC) but are very rare in sporadic breast cancer, and overall loss of SDHx gene expression is a signature of DTC PMID: 25694510
    41. SDHB immunohistochemistry alone may be misleading in excluding tumors other than gastrointestinal stromal tumors PMID: 25205505
    42. SDHB plays a key role in cell proliferation, invasion, migration, and apoptosis of human ovarian carcinoma. PMID: 25491408
    43. A novel mutation in SDH-B gene in an Iranian girl with paraganglioma and her family. PMID: 25402382
    44. Found an association between germline mutation of SDHB and a familial paraganglioma/pheochromocytoma plus pituitary adenoma. PMID: 25695889
    45. Succinate dehydrogenase B expression in clear cell papillary renal cell carcinoma with high nucleolar grade (G3-G4) is significantly associated with survival, indicating it may be both a diagnostic and prognostic marker in renal cell carcinoma PMID: 25827535
    46. two main Dutch SDHB founder mutations do not differ in clinical expression and result in a relatively mild phenotype of paragangliomas PMID: 25047027
    47. SDHB mutation testing has no utility in adrenaline producing pheochromocytomas, but is indicated in patients with metastatic disease. PMID: 25371406
    48. demonstrate that although SDHB mutations significantly downregulate both mitochondrial and cytoplasmic cellular metabolism, these mutations are associated to an upregulation of some cellular functions, such as growth rate and invasiveness PMID: 24595825
    49. Low SDHB expression is associated with metastasis in phaeochromocytoma and paraganglioma. PMID: 24521857
    50. Succinate Dehydrogenase mutations are associated with pheochromocytoma and paraganglioma. PMID: 24500761

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  • 相关疾病：
    Pheochromocytoma (PCC); Paragangliomas 4 (PGL4); Paraganglioma and gastric stromal sarcoma (PGGSS); Cowden syndrome 2 (CWS2)
  • 亚细胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    Succinate dehydrogenase/fumarate reductase iron-sulfur protein family
  • 数据库链接：

    HGNC: 10681

    OMIM: 115310

    KEGG: hsa:6390

    STRING: 9606.ENSP00000364649

    UniGene: Hs.465924