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