SDHB Recombinant Monoclonal Antibody

說明書

中文名稱：

SDHB重組抗體
貨號：

CSB-RA987662A0HU
規格：

￥1320
圖片：
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：

產品詳情
產品評價
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靶點詳情

產品詳情

產品描述：

SDHB Recombinant Monoclonal Antibody（CSB-RA987662A0HU）靶向琥珀酸脫氫酶復合體B亞基（SDHB），該蛋白是線粒體呼吸鏈Ⅱ復合體的核心組分，參與三羧酸循環和電子傳遞過程，其功能異常與多種代謝性疾病及腫瘤發生相關。本產品為重組單克隆抗體，具有高特異性和批次一致性，經嚴格驗證可應用于ELISA、Western Blot（WB）、免疫組化（IHC）及流式細胞術（FC）等多種實驗場景。在WB中可清晰檢測目標蛋白，推薦稀釋比例為1:500-1:5000；IHC實驗顯示對石蠟包埋組織切片中SDHB的定位敏感性高，建議使用1:50-1:200稀釋；FC分析細胞表面或胞內表達時推薦1:20-1:200稀釋。該抗體適用于研究SDHB在腫瘤代謝重編程、線粒體功能障礙及遺傳性副神經節瘤等疾病中的作用機制，為探索能量代謝調控、癌癥生物學及病理模型構建提供可靠工具，尤其適用于需要高精度檢測SDHB蛋白表達水平的細胞生物學與分子病理學研究。
Uniprot No.：

P21912
基因名：

SDHB
別名：

Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial (EC 1.3.5.1) (Iron-sulfur subunit of complex II) (Ip), SDHB, SDH SDH1
反應種屬：

Human, Mouse, Rat
免疫原：

A synthesized peptide derived from human SDHB
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

7D12
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
產品提供形式：

Liquid
應用范圍：

ELISA, WB, IHC, FC

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:5000
IHC	1:50-1:200
FC	1:20-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
Flow cytometry (FC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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).
基因功能參考文獻：
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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相關疾病：

Pheochromocytoma (PCC); Paragangliomas 4 (PGL4); Paraganglioma and gastric stromal sarcoma (PGGSS); Cowden syndrome 2 (CWS2)
亞細胞定位：

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
蛋白家族：

Succinate dehydrogenase/fumarate reductase iron-sulfur protein family
數據庫鏈接：

HGNC: 10681

OMIM: 115310

KEGG: hsa:6390

STRING: 9606.ENSP00000364649

UniGene: Hs.465924

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