SDHB Antibody
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中文名稱：SDHB兔多克隆抗體
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貨號：CSB-PA020907GA01HU
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規格：￥3,900
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：CWS2 antibody; DHSB_HUMAN antibody; FLJ92337 antibody; Ip antibody; Iron sulfur subunit antibody; Iron sulfur subunit of complex II antibody; Iron-sulfur subunit of complex II antibody; mitochondrial antibody; PGL 4 antibody; PGL4 antibody; SDH 1 antibody; SDH antibody; SDH1 antibody; SDH2 antibody; SDH2, homolog of antibody; SdhB antibody; SDHIP antibody; Succinate dehydrogenase [ubiquinone] iron sulfur protein mitochondrial antibody; Succinate dehydrogenase [ubiquinone] iron sulfur subunit antibody; Succinate dehydrogenase [ubiquinone] iron-sulfur subunit antibody; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial antibody; Succinate Dehydrogenase 1 Iron Sulfur Subunit antibody; Succinate Dehydrogenase 2, S. cerevisiae, homolog of antibody; Succinate dehydrogenase complex iron sulfur subunit B antibody; Succinate dehydrogenase complex subunit B iron sulfur antibody; Succinate Dehydrogenase Complex Subunit B Iron Sulfur Protein antibody; succinate dehydrogenase complex, subunit B, iron sulfur (Ip) antibody; Succinate dehydrogenase iron sulfur protein antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Human SDHB
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產品提供形式：Liquid
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應用范圍：ELISA,WB
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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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).
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基因功能參考文獻：
- Mutation is detected in the SDHB gene both mother and a child PMID: 30178964
- newly identified IVS2-2A>C mutation in SDHB is responsible for Hereditary Pheochromocytoma/Paraganglioma Syndrome. PMID: 29925701
- 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
- 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
- Germline SDHB mutation is associated with Pheochromocytoma and Paraganglioma. PMID: 28884434
- 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
- Results identified a high frequency of germline mutation in SDHB gene in patients presenting with bladder paraganglioma. PMID: 27542510
- automation, reproducibility, and cost efficiency of SDHB IHC offer advantages over the labor-intensive histochemical method requiring frozen sections PMID: 27556822
- 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
- Mutation in the SDHB gene is associated with mediastinal paraganglioma. PMID: 28891197
- We report for the first time the presence of both TFE3 translocation and SDHB mutation in the same renal cell carcinoma tumor. PMID: 27910947
- 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
- miR-142-5p up-regulation in colorectal cancer probably facilitates generation of aerobic glycolysis by reducing SDHB expression. PMID: 28622713
- SDHB mutation is associated with metastatic pheochromocytoma. PMID: 28409892
- 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
- LASS5 interacts with SDHB and synergistically represses p53 and p21 activity. PMID: 27280497
- A novel SDHB germline mutation in a paraganglioma PMID: 26833045
- SDHB-related tumours are picked up as early as 2 years after initial negative surveillance scan PMID: 27678251
- miR-183 cluster (miR-96/182/183) are related to clinical parameters and SDHB expression in pheochromocytomas. PMID: 28412207
- 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
- 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
- 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
- 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
- SDHB mutation status and primary tumor size are predictive in patient outcome diagnosed with pheochromocytoma and abdominal paraganglioma. PMID: 27839933
- 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
- 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
- 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
- Of which 4 SDHB and 2 TMEM127 mutations were novel. PMID: 26960314
- 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
- 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
- that SDHAF1 contributes to iron-sulfur (Fe-S) cluster incorporation into the Fe-S subunit of CII, SDHB. PMID: 26749241
- 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
- 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
- 15 YEARS OF PARAGANGLIOMA: Genetics and mechanism of pheochromocytoma-paraganglioma syndromes characterized by germline SDHB and SDHD mutations PMID: 26113606
- Study reveals new insights into the effects of SDHB mutations and the power of structural modelling in predicting biological consequences. PMID: 25972245
- for the first time, we show a high correlation between urinary bladder paragangliomas and SDHB mutations PMID: 25683602
- 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
- Germline mutation in the SDHB gene is the only reliable predictor of malignant Paragangliomas. PMID: 24973967
- Report direct correlation between the presence of an SDHB mutation, whether germline or somatic, and negative SDHB immunohistochemical staining in bladder paragangliomas. PMID: 26457353
- 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
- SDHB immunohistochemistry alone may be misleading in excluding tumors other than gastrointestinal stromal tumors PMID: 25205505
- SDHB plays a key role in cell proliferation, invasion, migration, and apoptosis of human ovarian carcinoma. PMID: 25491408
- A novel mutation in SDH-B gene in an Iranian girl with paraganglioma and her family. PMID: 25402382
- Found an association between germline mutation of SDHB and a familial paraganglioma/pheochromocytoma plus pituitary adenoma. PMID: 25695889
- 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
- two main Dutch SDHB founder mutations do not differ in clinical expression and result in a relatively mild phenotype of paragangliomas PMID: 25047027
- SDHB mutation testing has no utility in adrenaline producing pheochromocytomas, but is indicated in patients with metastatic disease. PMID: 25371406
- 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
- Low SDHB expression is associated with metastasis in phaeochromocytoma and paraganglioma. PMID: 24521857
- Succinate Dehydrogenase mutations are associated with pheochromocytoma and paraganglioma. PMID: 24500761
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相關疾病：Pheochromocytoma (PCC); Paragangliomas 4 (PGL4); Paraganglioma and gastric stromal sarcoma (PGGSS); Cowden syndrome 2 (CWS2)
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亞細胞定位：Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
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蛋白家族：Succinate dehydrogenase/fumarate reductase iron-sulfur protein family
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數據庫鏈接：
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