SDHC

Summary

Gene Symbol: SDHC
Description: succinate dehydrogenase complex subunit C
Alias: CYB560, CYBL, PGL3, QPS1, SDH3, succinate dehydrogenase cytochrome b560 subunit, mitochondrial, cytochrome B large subunit of complex II, integral membrane protein CII-3b, large subunit of cytochrome b, succinate dehydrgenase cytochrome b, succinate dehydrogenase 3, integral membrane subunit, succinate dehydrogenase complex subunit C integral membrane protein 15kDa, succinate dehydrogenase complex, subunit C, integral membrane protein, 15kD, succinate-ubiquinone oxidoreductase cytochrome B large subunit
Species: human
Products:     SDHC

Top Publications

  1. Oostveen F, Au H, Meijer P, Scheffler I. A Chinese hamster mutant cell line with a defect in the integral membrane protein CII-3 of complex II of the mitochondrial electron transport chain. J Biol Chem. 1995;270:26104-8 pubmed
    ..The gene for CII-3 was mapped on human chromosome 1, and because it is also found on minichromosomes characterized by our laboratory, we can localize it on the short arm within 1-2 megabases from the centromere...
  2. Niemann S, Muller U. Mutations in SDHC cause autosomal dominant paraganglioma, type 3. Nat Genet. 2000;26:268-70 pubmed
    ..Between 10% and 50% of cases are familial and are transmitted as autosomal dominant traits with incomplete and age-dependent penetrance...
  3. Astuti D, Latif F, Dallol A, Dahia P, Douglas F, George E, et al. Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. Am J Hum Genet. 2001;69:49-54 pubmed
    ..The gene products of two components of succinate dehydrogenase, SDHC and SDHD, anchor the gene products of two other components, SDHA and SDHB, which form the catalytic core, to the ..
  4. Baysal B, Willett Brozick J, Lawrence E, Drovdlic C, Savul S, McLeod D, et al. Prevalence of SDHB, SDHC, and SDHD germline mutations in clinic patients with head and neck paragangliomas. J Med Genet. 2002;39:178-83 pubmed
    ..Germline mutations in the mitochondrial complex II genes, SDHB, SDHC, and SDHD, cause hereditary paraganglioma (PGL)...
  5. Niemann S, Muller U, Engelhardt D, Lohse P. Autosomal dominant malignant and catecholamine-producing paraganglioma caused by a splice donor site mutation in SDHC. Hum Genet. 2003;113:92-4 pubmed
    Mutations in SDHC cause autosomal dominant paraganglioma, type 3 (PGL3), and have to date been demonstrated in only one family...
  6. Baysal B, Willett Brozick J, Filho P, Lawrence E, Myers E, Ferrell R. An Alu-mediated partial SDHC deletion causes familial and sporadic paraganglioma. J Med Genet. 2004;41:703-9 pubmed
  7. Mhatre A, Li Y, Feng L, Gasperin A, Lalwani A. SDHB, SDHC, and SDHD mutation screen in sporadic and familial head and neck paragangliomas. Clin Genet. 2004;66:461-6 pubmed
    Mutations within three genes, SDHB, SDHC, and SDHD, encoding distinct subunits of a hetero-oligomeric protein known as the mitochondrial complex II, a component of the mitochondrial electron transport chain and the Krebs cycle have been ..
  8. Sharma S, Wang J, Cortes Gomez E, TAGGART R, Baysal B. Mitochondrial complex II regulates a distinct oxygen sensing mechanism in monocytes. Hum Mol Genet. 2017;26:1328-1339 pubmed publisher
    ..Accordingly, compound germline heterozygosity of mouse Sdhb/Sdhc/Sdhd null alleles blunts chronic hypoxia-induced increases in hemoglobin levels, an adaptive response mainly ..
  9. Pasini B, Stratakis C. SDH mutations in tumorigenesis and inherited endocrine tumours: lesson from the phaeochromocytoma-paraganglioma syndromes. J Intern Med. 2009;266:19-42 pubmed publisher
    ..caused by germline mutations in three genes encoding subunits of the succinate dehydrogenase (SDH) enzyme (SDHB, SDHC and SDHD) thus opening an unexpected connection between mitochondrial tumour suppressor genes and neural crest-..

More Information

Publications121 found, 100 shown here

  1. Papathomas T, Oudijk L, Persu A, Gill A, van Nederveen F, Tischler A, et al. SDHB/SDHA immunohistochemistry in pheochromocytomas and paragangliomas: a multicenter interobserver variation analysis using virtual microscopy: a Multinational Study of the European Network for the Study of Adrenal Tumors (ENS@T). Mod Pathol. 2015;28:807-21 pubmed publisher
    ..If SDH molecular genetic analysis fails to detect a mutation in SDHB-immunonegative tumor, SDHC promoter methylation and/or VHL/NF1 testing with the use of targeted next-generation sequencing is advisable.
  2. Benn D, Robinson B, Clifton Bligh R. 15 YEARS OF PARAGANGLIOMA: Clinical manifestations of paraganglioma syndromes types 1-5. Endocr Relat Cancer. 2015;22:T91-103 pubmed publisher
    ..PGL4 syndrome (SDHB) is notable for an increased risk of malignant PGL or PC. PGL3 syndrome (SDHC) and PGL5 syndrome (SDHA) are less common and appear to be associated with lower penetrance of tumour development...
  3. Lussey Lepoutre C, Buffet A, Gimenez Roqueplo A, Favier J. Mitochondrial Deficiencies in the Predisposition to Paraganglioma. Metabolites. 2017;7: pubmed publisher
    ..intriguingly caused by mutations in genes encoding tricarboxylic acid enzymes, namely SDHA, SDHB, SDHC, SDHD, and SDHAF2 genes, encoding succinate dehydrogenase and its assembly protein, FH ..
  4. Janeway K, Kim S, Lodish M, Nose V, Rustin P, Gaal J, et al. Defects in succinate dehydrogenase in gastrointestinal stromal tumors lacking KIT and PDGFRA mutations. Proc Natl Acad Sci U S A. 2011;108:314-8 pubmed publisher
    ..patients (12%) with WT GIST without a personal or family history of paraganglioma had germline mutations in SDHB or SDHC. WT GISTs lacking somatic mutations or deletions in SDH subunits had either complete loss of or substantial ..
  5. Hoekstra A, Devilee P, Bayley J. Models of parent-of-origin tumorigenesis in hereditary paraganglioma. Semin Cell Dev Biol. 2015;43:117-124 pubmed publisher
    ..the genes related to mitochondrial succinate dehydrogenase (SDH) including SDHA, SDHB, SDHC, SDHD and the assembly factor SDHAF2...
  6. Hernández K, Ezzat S, Morel C, Swallow C, Otremba M, Dickson B, et al. Familial pheochromocytoma and renal cell carcinoma syndrome: TMEM127 as a novel candidate gene for the association. Virchows Arch. 2015;466:727-32 pubmed publisher
    Germline mutations in Von Hippel-Lindau (VHL), succinate dehydrogenase subunit B (SDHB), SDHC, and SDHD have been detected in individuals with synchronous or metachronous pheochromocytoma/paraganglioma (PHEO/PGL) and renal cell carcinoma (..
  7. Sagong B, Seo Y, Lee H, Kim M, Kim U, Moon I. A mutation of the succinate dehydrogenase B gene in a Korean family with paraganglioma. Fam Cancer. 2016;15:601-6 pubmed publisher
    ..Germline mutations in genes coding for succinate dehydrogenase (SDH) subunits D, B, and C (SDHD, SDHB, SDHC) are found in almost all familial PGL patients...
  8. Sun Z, Wang B, Liu Y, Liu X, Mi Y, Gu M, et al. Genome organization and transcriptional regulation of Adenosine Deaminase Acting on RNA gene 1 (ADAR1) in grass carp (Ctenopharyngodon idella). Dev Comp Immunol. 2015;50:98-105 pubmed publisher
    ..Co-transfection of pcDNA3.1-IRF1 (or pcDNA3.1-IRF3) with pGL3-CiADAR1 into C...
  9. Bardella C, Pollard P, Tomlinson I. SDH mutations in cancer. Biochim Biophys Acta. 2011;1807:1432-43 pubmed publisher
    The SDHA, SDHB, SDHC, SDHD genes encode the four subunits of succinate dehydrogenase (SDH; mitochondrial complex II), a mitochondrial enzyme involved in two essential energy-producing metabolic processes of the cell, the Krebs cycle and ..
  10. Bahougne T, Romanet P, Mohamed A, Caselles K, Cuny T, Barlier A, et al. Functioning Mediastinal Paraganglioma Associated with a Germline Mutation of von Hippel-Lindau Gene. J Clin Med. 2018;7: pubmed publisher
    ..Mutations in SDHA, SDHB, SDHC, SDHD, RET, SDHAF2, TMEM127, MAX, NF1, FH, MDH2, and ..
  11. Wang B, Liu Y, Sun Z, Liu X, Mi Y, Liu D, et al. A splicing isoform of Ctenopharyngodon idella ADAR1 (CiADAR1-like): Genome organization, tissue specific expression and transcriptional regulation. Fish Shellfish Immunol. 2015;47:535-44 pubmed publisher
    ..In vivo, Co-transfection of pcDNA3.1-IRF1 (or pcDNA3.1-IRF3) with pGL3-CiADAR1-like promoter into CIK cells showed that both IRF1 and IRF3 significantly increased the luciferase activity,..
  12. Rednam S, Erez A, Druker H, Janeway K, Kamihara J, Kohlmann W, et al. Von Hippel-Lindau and Hereditary Pheochromocytoma/Paraganglioma Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood. Clin Cancer Res. 2017;23:e68-e75 pubmed publisher
    ..majority of patients with HPP syndrome harbor an underlying variant in one of the SHDx genes (SDHA, SDHB, SDHC, SDHD, SDHA, and SDHAF2), although other genes also have been described (MAX and TMEM127)...
  13. Jasperson K, Kohlmann W, Gammon A, Slack H, Buchmann L, Hunt J, et al. Role of rapid sequence whole-body MRI screening in SDH-associated hereditary paraganglioma families. Fam Cancer. 2014;13:257-65 pubmed publisher
    ..sensitivity for SDH-related tumors than biochemical testing in patients undergoing screening due to their SDHB or SDHC mutation status...
  14. Yadav N, Kumar S, Kumar R, Srivastava P, Sun L, Rapali P, et al. Mechanism of neem limonoids-induced cell death in cancer: Role of oxidative phosphorylation. Free Radic Biol Med. 2016;90:261-71 pubmed publisher
    ..Complex II-deficiency due to the loss of succinate dehydrogenase complex subunit C (SDHC) robustly decreased caspase activation, apoptosis, and cell death...
  15. Liu X, Huang K, Hou Q, Sun Z, Wang B, Lin G, et al. Identification and characterization of a constitutively expressed Ctenopharyngodon idella ADAR1 splicing isoform (CiADAR1a). Dev Comp Immunol. 2016;63:10-7 pubmed publisher
    ..1 eukaryotic expression vectors with IRF1 and IRF3 and co-transfected them with pGL3-CiADAR1a promoter into CIK cells...
  16. Huang K, Qi G, Sun Z, Liu X, Xu X, Wang H, et al. Ctenopharyngodon idella IRF2 and ATF4 down-regulate the transcriptional level of PRKRA. Fish Shellfish Immunol. 2017;64:155-164 pubmed publisher
    ..Recombinant plasmid of pGL3-PRKRAPro was constructed and transiently co-transfected into CIK cells with pcDNA3.1-CiIRF2 and pcDNA3...
  17. Wang H, Zhu Y, Xu X, Wang X, Hou Q, Xu Q, et al. Ctenopharyngodon idella NF-?B subunit p65 modulates the transcription of I?B? in CIK cells. Fish Shellfish Immunol. 2016;54:564-72 pubmed publisher
    ..In vivo, the cotransfection of pcDNA3.1-Cip65 (or pcDNA3.1-Cip65-?C, pcDNA3.1-Cip65-?N respectively) with pGL3-CiI?B? and pRL-TK renilla luciferase plasmid into CIK cells showed that pcDNA3.1-Cip65 rather than pcDNA3...
  18. Takahata S, Kubota N, Takei Masuda N, Yamada T, Maeda M, Alshahni M, et al. Mechanism of Action of ME1111, a Novel Antifungal Agent for Topical Treatment of Onychomycosis. Antimicrob Agents Chemother. 2016;60:873-80 pubmed publisher
    ..resistant mutants tested harbored a mutation in one of the subunits of succinate dehydrogenase (SdhB, SdhC, or SdhD)...
  19. Gill A, Hes O, Papathomas T, Šedivcová M, Tan P, Agaimy A, et al. Succinate dehydrogenase (SDH)-deficient renal carcinoma: a morphologically distinct entity: a clinicopathologic series of 36 tumors from 27 patients. Am J Surg Pathol. 2014;38:1588-602 pubmed publisher
    ..who underwent genetic testing for mutation in the SDH subunits demonstrated germline mutations (16 in SDHB and 1 in SDHC). Nine of 27 (33%) patients developed metastatic disease, 2 of them after prolonged follow-up (5.5 and 30 y)...
  20. Hayashi S, Itoh K, Suyama K. Genes of Bacillus subtilis 168 that Support Growth of the Cyanobacterium, Synechococcus leopoliensis CCAP1405/1 on Agar Media. Microb Ecol. 2015;70:849-52 pubmed publisher
    ..leopoliensis CCAP1405/1 on agar media: yxeO and yufO (transporter), yxdK (histidine kinase), sdhC (succinate dehydrogenase), yvgQR (sulfite reductase), acoB (acetoin dehydrogenase), yusE (thioredoxin), yrdA (..
  21. Remacha L, Comino Mendez I, Richter S, Contreras L, Currás Freixes M, Pita G, et al. Targeted Exome Sequencing of Krebs Cycle Genes Reveals Candidate Cancer-Predisposing Mutations in Pheochromocytomas and Paragangliomas. Clin Cancer Res. 2017;23:6315-6324 pubmed publisher
    ..Array methylation-based analysis uncovered a somatic epigenetic mutation in SDHC in a patient with multiple pheochromocytomas and a gastrointestinal stromal tumor...
  22. Jiang R, Lonnerdal B. Cloning and characterization of the human lactoferrin receptor gene promoter. Biometals. 2018;31:357-368 pubmed publisher
    ..A series of deletions of 5'-flanking sequences of the human LfR gene were cloned into a promoter-less pGL3 luciferase reporter and transiently transfected into an intestinal enterocyte model (Caco-2 cells)...
  23. Maiti B, Kamra M, Karande A, Bhattacharya S. Transfection efficiencies of α-tocopherylated cationic gemini lipids with hydroxyethyl bearing headgroups under high serum conditions. Org Biomol Chem. 2018;16:1983-1993 pubmed publisher
    ..Relatively good binding efficiency and easy release of pDNA (pGL3) were also observed with DOPE-TH8S (2 : 1) in the ethidium bromide (EB)-exclusion and re-intercalation assay, ..
  24. Dénes J, Swords F, Rattenberry E, Stals K, Owens M, Cranston T, et al. Heterogeneous genetic background of the association of pheochromocytoma/paraganglioma and pituitary adenoma: results from a large patient cohort. J Clin Endocrinol Metab. 2015;100:E531-41 pubmed publisher
    ..Outcomes included genetic screening and clinical characteristics. Eleven germline mutations (five SDHB, one SDHC, one SDHD, two VHL, and two MEN1) and four variants of unknown significance (two SDHA, one SDHB, and one SDHAF2) ..
  25. Belt K, Van Aken O, Murcha M, Millar A, Huang S. An Assembly Factor Promotes Assembly of Flavinated SDH1 into the Succinate Dehydrogenase Complex. Plant Physiol. 2018;177:1439-1452 pubmed publisher
    ..We conclude that SDHAF4 acts on FAD-SDH1 and promotes its assembly with SDH2, thereby stabilizing SDH2 and enabling its full assembly with SDH3/SDH4 to form the SDH complex.
  26. Courage C, Jackson C, Hahn D, Euro L, Nuoffer J, Gallati S, et al. SDHA mutation with dominant transmission results in complex II deficiency with ocular, cardiac, and neurologic involvement. Am J Med Genet A. 2017;173:225-230 pubmed publisher
    ..Of the four nuclear encoded proteins composing complex II (SDHA, SDHB, SDHC, SDHD) and currently known assembly factors SDHAF1 and SDHAF2 mainly recessively inherited mutations have been ..
  27. Gupta S, Zhang J, Rivera M, Erickson L. Urinary Bladder Paragangliomas: Analysis of Succinate Dehydrogenase and Outcome. Endocr Pathol. 2016;27:243-52 pubmed publisher
    ..Interestingly, of these four patients, two had a documented mutation of SDHB, one patient had a SDHC mutation, and another patient had a history of familial disease without mutation analysis being performed...
  28. Wang X, Zhang T, Mao H, Mi Y, Zhong B, Wei L, et al. Grass carp (Ctenopharyngodon idella) ATF6 (activating transcription factor 6) modulates the transcriptional level of GRP78 and GRP94 in CIK cells. Fish Shellfish Immunol. 2016;52:65-73 pubmed publisher
    ..Recombinant plasmids of pGL3-GRP78P and pGL3-CiGRP94P were constructed and transiently co-transfected with pcDNA3.1-CiATF6 (pcDN3...
  29. Aldera A, Govender D. Gene of the month: SDH. J Clin Pathol. 2017;: pubmed publisher
    ..Germline mutations in SDHA are associated with Leigh syndrome. Mutations in SDHB, SDHC and SDHD are found in an increasing number of neoplasms, most notably paragangliomas and wild-type ..
  30. Zanotti S, Canalis E. Interleukin 6 mediates selected effects of Notch in chondrocytes. Osteoarthritis Cartilage. 2013;21:1766-73 pubmed publisher
    ..of Notch action on Il6 expression, cells were transfected with a fragment of the Il6 promoter or control vector pGL3, or transcriptionally arrested with 5,6-dichloro-1-β-d-ribofuranosylbenzimidazole...
  31. Bonache S, Martínez J, Fernández M, Bassas L, Larriba S. Single nucleotide polymorphisms in succinate dehydrogenase subunits and citrate synthase genes: association results for impaired spermatogenesis. Int J Androl. 2007;30:144-52 pubmed
    Evaluation of the possible implication of the SDHA, SDHB, SDHC, SDHD and CS genes in non-obstructive male infertility was performed on the basis that sperm concentration in the ejaculate has been previously correlated with nuclear-encoded ..
  32. Xu X, Lai Q, Gu M, Liu D, Hou Q, Liu X, et al. Fish IRF3 up-regulates the transcriptional level of IRF1, IRF2, IRF3 and IRF7 in CIK cells. Fish Shellfish Immunol. 2015;47:978-85 pubmed publisher
    ..Then, CIK cells were co-transfected with pcDNA3.1-CiIRF3, pGL3-promotor (pGL3-CiIRF1, pGL3-CiIRF2, pGL3-CiIRF3, pGL3-CiIRF7) and luciferase reporter vector respectively...
  33. Xiong J, Hu Z, Wang T, Xu X, Liu J, Wu P, et al. RUNX2 controls human IPO8 basal transcription in Saos-2 cells. Mol Med Rep. 2016;14:1418-24 pubmed publisher
    ..The 5' flanking region of the IPO8 gene, which is ~3,300 bp in length, was cloned and inserted into the pGL3?basic luciferase reporter vector...
  34. Cascon A, Pita G, Burnichon N, Landa I, López Jiménez E, Montero Conde C, et al. Genetics of pheochromocytoma and paraganglioma in Spanish patients. J Clin Endocrinol Metab. 2009;94:1701-5 pubmed publisher
    ..Two hundred thirty-seven nonrelated probands were analyzed for the major susceptibility genes: VHL, RET, SDHB, SDHC, and SDHD...
  35. Kuroda N, Yorita K, Nagasaki M, Harada Y, Ohe C, Jeruc J, et al. Review of succinate dehydrogenase-deficient renal cell carcinoma with focus on clinical and pathobiological aspects. Pol J Pathol. 2016;67:3-7 pubmed
    ..Succinate dehydrogenase (SDH) is an enzyme complex composed of four protein subunits (SDHA, SDHB, SDHC and SDHD). The tumor which presents this enzyme mutation accounts for 0.05 to 0.2% of all renal carcinomas...
  36. Liu J, Wei Y, Huang L, Wang Y, Chen D, Wu H, et al. Functional characterization of a new promoter isolated from torque teno sus virus 1. Arch Virol. 2016;161:303-6 pubmed publisher
    ..We used a dual-luciferase reporter system, involving pGL3-Basic and pRL-TK, to identify the promoter of TTSuV1 ORF1...
  37. Dai L, Chu X, Lu F, Xu R. Detection of four polymorphisms in 5' upstream region of PNPLA2 gene and their associations with economic traits in pigs. Mol Biol Rep. 2016;43:1305-1313 pubmed
    ..05) than those with diplotype H2H2 and H3H3. Luciferase assay indicated pGL3-basic-H2 had the highest activity and pGL3-basic-H1 had the lowest activity in driving reporter gene transcription ..
  38. Schikowsky C, Senkler J, Braun H. SDH6 and SDH7 Contribute to Anchoring Succinate Dehydrogenase to the Inner Mitochondrial Membrane in Arabidopsis thaliana. Plant Physiol. 2017;173:1094-1108 pubmed publisher
    ..Another subcomplex (IIb) is composed of the SDH3, SDH4, SDH6, and SDH7 subunits...
  39. Mascarello J, Soderberg K, Scheffler I. Assignment of a gene for succinate dehydrogenase to human chromosome 1 by somatic cell hybridization. Cytogenet Cell Genet. 1980;28:121-35 pubmed
    ..We present in one of the two structural genes for the 70,000 and 30,000 dalton peptides, respectively, which constitute succinate dehydrogenase. One of these two genes is therefore mapped on human chromosome 1. ..
  40. Hu S, Liu L, Chang E, Wang J, Raufman J. Butyrate inhibits pro-proliferative miR-92a by diminishing c-Myc-induced miR-17-92a cluster transcription in human colon cancer cells. Mol Cancer. 2015;14:180 pubmed publisher
    ..Regulation of C13orf25 promoter activity by butyrate was analyzed by luciferase reporter assay using modified pGL3 constructs containing a wild-type or mutated c-Myc binding site...
  41. De Sousa S, McCabe M, Wu K, Roscioli T, Gayevskiy V, Brook K, et al. Germline variants in familial pituitary tumour syndrome genes are common in young patients and families with additional endocrine tumours. Eur J Endocrinol. 2017;176:635-644 pubmed publisher
    ..of rare variants in AIP, CDKN1B, MEN1, PRKAR1A, SDHA, SDHB, SDHC and SDHD...
  42. Khadilkar K, Sarathi V, Kasaliwal R, Pandit R, Goroshi M, Shivane V, et al. Genotype-phenotype correlation in paediatric pheochromocytoma and paraganglioma: a single centre experience from India. J Pediatr Endocrinol Metab. 2017;30:575-581 pubmed publisher
    ..index PCC/PGL patients underwent genetic testing for five PCC/PGL susceptibility genes (RET, VHL, SDHB, SDHD and SDHC) and were evaluated for clinical diagnosis of neurofibromatosis type1 (NF1)...
  43. Pathak B, Breed A, Deshmukh P, Mahale S. Androgen receptor mediated epigenetic regulation of CRISP3 promoter in prostate cancer cells. J Steroid Biochem Mol Biol. 2018;181:20-27 pubmed publisher
    ..to understand the regulation of CRISP3 expression, various overlapping fragments of CRISP3 promoter were cloned in pGL3 luciferase reporter vector...
  44. Lussey Lepoutre C, Bellucci A, Morin A, Buffet A, Amar L, Janin M, et al. In Vivo Detection of Succinate by Magnetic Resonance Spectroscopy as a Hallmark of SDHx Mutations in Paraganglioma. Clin Cancer Res. 2016;22:1120-9 pubmed publisher
    ..Detection of succinate by (1)H-MRS is a highly specific and sensitive hallmark of SDHx mutations. This noninvasive approach is a simple and robust method allowing in vivo detection of the major biomarker of SDHx-mutated tumors. ..
  45. Yukina M, Troshina E, Beltsevich D. [Hereditary pheochromocytoma-associated syndromes. Part 1]. Ter Arkh. 2015;87:102-105 pubmed publisher
    ..There have been the most investigated NF, RET, VHL, SDHD, SDHC, and SDHB gene mutations...
  46. Wang X, Tang S, Song F, Chen C, Guo X, Shen X. Bmo-miR-2758 Targets BmFMBP-1 (Lepidoptera: Bombycidae) and Suppresses Its Expression in BmN Cells. J Insect Sci. 2016;16: pubmed publisher
    ..regulation function of bmo-miR-2758 on BmFMBP-1, recombinant plasmids pcDNA3 [ie1-egfp-pri-bmo-miR-2758-SV40] and pGL3 [A3-luc-FMBP-1 3' UTR-SV40] were constructed and co-transfected in BmN cells...
  47. Zhu S, Soutto M, Chen Z, Peng D, Romero Gallo J, Krishna U, et al. Helicobacter pylori-induced cell death is counteracted by NF-?B-mediated transcription of DARPP-32. Gut. 2017;66:761-762 pubmed publisher
    ..regulation of DARPP-32, we cloned a 3019?bp of the DARPP-32 promoter into the luciferase reporter (pGL3-Luc). Both H...
  48. Lin J, Zhang Y, Wang H, Chang J, Wei L, Cao L, et al. Genetic Polymorphisms in the Apoptosis-Associated Gene CASP3 and the Risk of Lung Cancer in Chinese Population. PLoS ONE. 2016;11:e0164358 pubmed publisher
    ..possible impact of 829 A>C SNP on CASP3 transcriptional activity, we detected the dual luciferase activity of PGL3-promoter vectors containing 829A or 829C alleles in lung cancer cell lines and found that report gene expressions ..
  49. Forcellini E, Boutin S, Lefebvre C, Shayhidin E, Boulanger M, Rhéaume G, et al. Synthesis and biological evaluation of novel quinazoline-4-piperidinesulfamide derivatives as inhibitors of NPP1. Eur J Med Chem. 2018;147:130-149 pubmed publisher
    ..A quinazoline-4-piperidine sulfamide compound (QPS1) has been described as a specific and non-competitive inhibitor of NPP1...
  50. Zhu W, Wang Z, Chai Y, Wang X, Chen D, Wu H. Germline mutations and genotype-phenotype associations in head and neck paraganglioma patients with negative family history in China. Eur J Med Genet. 2015;58:433-8 pubmed publisher
    ..Seven genes, Succinate dehydrogenase complex A,B,C,D (SDHA, SDHB, SDHC, SDHD), succinate dehydrogenase complex assembly factor 2 (SDHAF2), TMEM127 (transmembrane protein 127) and VHL (..
  51. Urbini M, Astolfi A, Indio V, Heinrich M, Corless C, Nannini M, et al. SDHC methylation in gastrointestinal stromal tumors (GIST): a case report. BMC Med Genet. 2015;16:87 pubmed publisher
    ..Gene expression analysis showed remarkable down-modulation of SDHC mRNA with respect to all other GIST samples, both SDHA-mutant and KIT/PDGFRA-mutant GIST...
  52. Prathibha Y, Senthilkumaran B. Expression of wnt4/5 during reproductive cycle of catfish and wnt5 promoter analysis. J Endocrinol. 2017;232:1-13 pubmed
    ..Promoter activity of wnt5 was analyzed by luciferase assays after transfecting progressive deletion constructs in pGL3 basic vector into the mammalian cell lines (HEK 293 and CHO)...
  53. Sui G, Liu Z, Wang T, Li X, Ding X, Liu X, et al. Promoter analysis of bovine cardiomyopathy-associated protein 1 gene. Genet Mol Res. 2016;15: pubmed publisher
    ..Thus, the CMYA1 promoter is muscle-specific. Thereafter, eight pGL3-basic vectors with various truncated CMYA1 promoter fragments were transfected into C2C12 cells, to identify the ..
  54. Yuan F, Sun R, Li L, Jin B, Wang Y, Liang Y, et al. A functional variant rs353292 in the flanking region of miR-143/145 contributes to the risk of colorectal cancer. Sci Rep. 2016;6:30195 pubmed publisher
    ..04). Moreover, the pGL3-rs353292T displayed a significantly lower luciferase activity than pGL3-rs353292C (P < 0.01)...
  55. Liu X, Li Y, Zhong Z, Tan H, Lin H, Chen S, et al. Incorporation of Viral Glycoprotein VSV-G Improves the Delivery of DNA by Erythrocyte Ghost into Cells Refractory to Conventional Transfection. Appl Biochem Biotechnol. 2017;181:748-761 pubmed publisher
    ..Plasmid DNA was condensed by various transfection reagents. A luciferase expression construct (pGL3-control) and a DsRed expression cassette (pCMV-DsRed) were used to evaluate the delivery efficiency of DNA/EG/VSV-G ..
  56. Chen W, Xu H, Chen X, Liu Z, Zhang W, Xia D. Functional and Activity Analysis of Cattle UCP3 Promoter with MRFs-Related Factors. Int J Mol Sci. 2016;17: pubmed publisher
    ..UCP3 can be regulated by muscle-related factors, deletion of cattle UCP3 promoter was amplified and cloned into pGL3-basic, pGL3-promoter and PEGFP-N3 vector, respectively, then transfected into C2C12 myoblasts cells and UCP3 ..
  57. Boyle K, Patinkin Z, Shapiro A, Bader C, Vanderlinden L, Kechris K, et al. Maternal obesity alters fatty acid oxidation, AMPK activity, and associated DNA methylation in mesenchymal stem cells from human infants. Mol Metab. 2017;6:1503-1516 pubmed publisher
    ..analysis of DNA methylation array revealed Ob-MSC hypermethylation in genes regulating FAO (PRKAG2, ACC2, CPT1A, SDHC) and corresponding lower mRNA content of these genes...
  58. Ozluk Y, Taheri D, Matoso A, Sanli O, Berker N, Yakirevich E, et al. Renal carcinoma associated with a novel succinate dehydrogenase A mutation: a case report and review of literature of a rare subtype of renal carcinoma. Hum Pathol. 2015;46:1951-5 pubmed publisher
    ..cell carcinoma (RCC) linked to germline mutation of succinate dehydrogenase subunits A, B, C, and D (SDHA, SDHB, SDHC, and SDHD, respectively) has been recently included as a provisional entity in the 2013 International Society of ..
  59. Bhowal A, Majumder S, Ghosh S, Basu S, Sen D, Roychowdhury S, et al. Pathway-based expression profiling of benign prostatic hyperplasia and prostate cancer delineates an immunophilin molecule associated with cancer progression. Sci Rep. 2017;7:9763 pubmed publisher
    ..LNCaP cells transfected with the FKBP52 promoter cloned in pGL3 basic showed increased luciferase activity which declined considerably when the promoter-construct was co-..
  60. Wang N, Tang H, Wang X, Wang W, Feng J. Homocysteine upregulates interleukin-17A expression via NSun2-mediated RNA methylation in T lymphocytes. Biochem Biophys Res Commun. 2017;493:94-99 pubmed publisher
    ..Furthermore, we analyzed the activity of pGL3-derived reporters bearing IL-17A mRNA fragments and found that methylation by NSun2 promoted the translation of IL-..
  61. Vandy F, Sisk G, Berguer R. Synchronous carotid body and thoracic paraganglioma associated with a germline SDHC mutation. J Vasc Surg. 2011;53:805-7 pubmed publisher
    ..Manifestations of those with a succinate dehydrogenase subunit C (SDHC) germline mutation have been almost exclusively reported as single head and neck paragangliomas (HNPGLs)...
  62. Aykin Burns N, Slane B, Liu A, Owens K, O Malley M, Smith B, et al. Sensitivity to low-dose/low-LET ionizing radiation in mammalian cells harboring mutations in succinate dehydrogenase subunit C is governed by mitochondria-derived reactive oxygen species. Radiat Res. 2011;175:150-8 pubmed
    ..in the gene coding for the mitochondrial electron transport chain protein succinate dehydrogenase subunit C (SDHC) demonstrate increases in steady-state levels of O?•- and H?O?...
  63. Shi W, Yan D, Zhao C, Xiao M, Wang Y, Ma H, et al. Inhibition of IL-6/STAT3 signaling in human cancer cells using Evista. Biochem Biophys Res Commun. 2017;491:159-165 pubmed publisher
    ..Evista attenuated STAT3 phosphorylation, decreased STAT3 transcriptional activity but much less in pGL3 and AP1 transcriptional luciferase activity, and decreased cell viability in vitro...
  64. Wohllk N, Thomas P, Huang E, Cote G. A human succinate-ubiquinone oxidoreductase CII-3 subunit gene ending in a polymorphic dinucleotide repeat is located within the sulfonylurea receptor (SUR) gene. Mol Genet Metab. 1998;65:187-90 pubmed
    ..The second gene CII-3b is expressed at a low level and contains a 102-bp internal deletion compared to CII-3 cDNA. These genes should prove valuable in the characterization of Complex II disorders...
  65. Brière J, Favier J, El Ghouzzi V, Djouadi F, Benit P, Gimenez A, et al. Succinate dehydrogenase deficiency in human. Cell Mol Life Sci. 2005;62:2317-24 pubmed
    ..Finally we stress the importance of SDH as a target and/or marker in a number of diseases and the need to better delineate the consequences of SDH deficiency in humans. ..
  66. Montani M, Schmitt A, Schmid S, Locher T, Saremaslani P, Heitz P, et al. No mutations but an increased frequency of SDHx polymorphisms in patients with sporadic and familial medullary thyroid carcinoma. Endocr Relat Cancer. 2005;12:1011-6 pubmed
    Germline mutations of the three succinate dehydrogenase subunits SDHB, SDHC and SDHD have recently been associated with familial pheochromocytoma and paraganglioma...
  67. Bayley J, van Minderhout I, Weiss M, Jansen J, Oomen P, Menko F, et al. Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma. BMC Med Genet. 2006;7:1 pubmed
    Germline mutations of the SDHD, SDHB and SDHC genes, encoding three of the four subunits of succinate dehydrogenase, are a major cause of hereditary paraganglioma and pheochromocytoma, and demonstrate that these genes are classic tumor ..
  68. Korpershoek E, Van Nederveen F, Dannenberg H, Petri B, Komminoth P, Perren A, et al. Genetic analyses of apparently sporadic pheochromocytomas: the Rotterdam experience. Ann N Y Acad Sci. 2006;1073:138-48 pubmed
    ..Although 7.5% germline mutations in a series of apparently sporadic PCCs are far less than the more than 20% reported in the literature, the figure is significant enough to consider germline mutation testing for each patient with PCC. ..
  69. Ricketts C, Woodward E, Killick P, Morris M, Astuti D, Latif F, et al. Germline SDHB mutations and familial renal cell carcinoma. J Natl Cancer Inst. 2008;100:1260-2 pubmed publisher
    ..investigated whether germline mutations in fumarate hydratase (FH) or succinate dehydrogenase subunit genes (SDHB, SDHC, SDHD) were associated with RCC susceptibility in 68 patients with no clinical evidence of an RCC susceptibility ..
  70. Cascon A, López Jiménez E, Landa I, Leskela S, Leandro García L, Maliszewska A, et al. Rationalization of genetic testing in patients with apparently sporadic pheochromocytoma/paraganglioma. Horm Metab Res. 2009;41:672-5 pubmed publisher
    ..135 apparently sporadic patients developing a single tumor for the five major susceptibility genes: VHL, RET, SDHB, SDHC, and SDHD. Fourteen percent of cases were found to harbor a germline mutation, and only 2...
  71. Gill A, Benn D, Chou A, Clarkson A, Muljono A, Meyer Rochow G, et al. Immunohistochemistry for SDHB triages genetic testing of SDHB, SDHC, and SDHD in paraganglioma-pheochromocytoma syndromes. Hum Pathol. 2010;41:805-14 pubmed publisher
    ..germline RET, Von Hippel-Lindau (VHL), neurofibromatosis type I (NF1), and succinate dehydrogenase subunits (SDHB, SDHC, and SDHD) mutations...
  72. Pantaleo M, Astolfi A, Urbini M, Nannini M, Paterini P, Indio V, et al. Analysis of all subunits, SDHA, SDHB, SDHC, SDHD, of the succinate dehydrogenase complex in KIT/PDGFRA wild-type GIST. Eur J Hum Genet. 2014;22:32-9 pubmed publisher
    ..In this study, we simultaneously sequenced the genome of all subunits, SDHA, SDHB, SDHC, and SDHD in a larger series of KIT/PDGFRA wild-type GIST in order to evaluate the frequency of the mutations and ..
  73. Killian J, Miettinen M, Walker R, Wang Y, Zhu Y, Waterfall J, et al. Recurrent epimutation of SDHC in gastrointestinal stromal tumors. Sci Transl Med. 2014;6:268ra177 pubmed publisher
    ..dSDH GISTs often harbor deleterious mutations in SDH subunit genes (SDHA, SDHB, SDHC, and SDHD, termed SDHx), but some are SDHx wild type (WT)...
  74. Rich T, Jackson M, Roman Gonzalez A, Shah K, Cote G, Jimenez C. Metastatic sympathetic paraganglioma in a patient with loss of the SDHC gene. Fam Cancer. 2015;14:615-9 pubmed publisher
    ..occasionally present with metastatic disease, while conversely malignant paragangliomas are rarely observed in SDHC carriers...
  75. Sato T, Akiyama M, Nakahama K, Seo S, Watanabe M, Tatsuzaki J, et al. A novel mode of stimulating platelet formation activity in megakaryocytes with peanut skin extract. J Nat Med. 2018;72:211-219 pubmed publisher
    ..levels, which are known to play an important role in thrombocytopoiesis and, based on the results of a pMARE-Luc (pGL3-MARE-luciferase) assay, had no influence on NF-E2 activation in Meg01 cells...
  76. Baysal B, Lawrence E, Ferrell R. Sequence variation in human succinate dehydrogenase genes: evidence for long-term balancing selection on SDHA. BMC Biol. 2007;5:12 pubmed
    ..In contrast, heterozygous mutations in SDHB, SDHC, and SDHD, the other SDH subunit genes, cause hereditary paraganglioma (PGL) tumors, which show constitutive ..
  77. Sharma B, Mount J, Karrow N. Functional characterization of a single nucleotide polymorphism in the 5' UTR region of the bovine toll-like receptor 4 gene. Dev Biol (Basel). 2008;132:331-6 pubmed
    ..validated by a dual luciferase reporter gene assay in which sequential progressive constructs were cloned into the pGL3 basic vector and transfected into bovine Mac-T cells...
  78. Gaal J, Burnichon N, Korpershoek E, Roncelin I, Bertherat J, Plouin P, et al. Isocitrate dehydrogenase mutations are rare in pheochromocytomas and paragangliomas. J Clin Endocrinol Metab. 2010;95:1274-8 pubmed publisher
    ..is caused by germline-inactivating mutations in the mitochondrial succinate dehydrogenase (SDH) genes SDHB, SDHC, SDHD, or SDHAF2, and VHL is the result of inactivating VHL gene mutations...
  79. Hermsen M, Sevilla M, Llorente J, Weiss M, Grimbergen A, Allonca E, et al. Relevance of germline mutation screening in both familial and sporadic head and neck paraganglioma for early diagnosis and clinical management. Cell Oncol. 2010;32:275-83 pubmed publisher
  80. Malinoc A, Sullivan M, Wiech T, Schmid K, Jilg C, Straeter J, et al. Biallelic inactivation of the SDHC gene in renal carcinoma associated with paraganglioma syndrome type 3. Endocr Relat Cancer. 2012;19:283-90 pubmed publisher
    ..RCC has never been described as a component of SDHC-associated PGL3...
  81. Weber A, Hoffmann M, Neumann H, Erlic Z. Somatic mutation analysis of the SDHB, SDHC, SDHD, and RET genes in the clinical assessment of sporadic and hereditary pheochromocytoma. Horm Cancer. 2012;3:187-92 pubmed publisher
    ..SDHB and VHL somatic mutation might play a role in the sporadic forms of tumor development. There is no clinical impact of mutation screening or LOH analysis of tumor specimens. ..
  82. Bickmann J, Sollfrank S, Schad A, Musholt T, Springer E, Miederer M, et al. Phenotypic variability and risk of malignancy in SDHC-linked paragangliomas: lessons from three unrelated cases with an identical germline mutation (p.Arg133*). J Clin Endocrinol Metab. 2014;99:E489-96 pubmed publisher
    ..b>SDHC mutations most frequently result in benign, nonfunctional head-and neck PGLs (HNPGLs)...
  83. Haller F, Moskalev E, Faucz F, Barthelmeß S, Wiemann S, Bieg M, et al. Aberrant DNA hypermethylation of SDHC: a novel mechanism of tumor development in Carney triad. Endocr Relat Cancer. 2014;21:567-77 pubmed publisher
    ..For the first time, we report on a recurrent aberrant dense DNA methylation at the gene locus of SDHC in tumors of patients with CT, which was not present in tumors of patients with CSS or PGL, or in sporadic GISTs ..
  84. Richter S, Klink B, Nacke B, de Cubas A, Mangelis A, Rapizzi E, et al. Epigenetic Mutation of the Succinate Dehydrogenase C Promoter in a Patient With Two Paragangliomas. J Clin Endocrinol Metab. 2016;101:359-63 pubmed publisher
    ..Epigenetic inactivation of the SDHC gene is a more recently discovered phenomenon, which so far has only been described in GISTs and PPGLs from ..
  85. Else T, Lerario A, Everett J, Haymon L, Wham D, Mullane M, et al. Adrenocortical carcinoma and succinate dehydrogenase gene mutations: an observational case series. Eur J Endocrinol. 2017;177:439-444 pubmed publisher
    ..All four ACC specimens were found to have truncating mutations in either SDHC or SDHA, while cases 1, 2 and 3 also had the mutations confirmed in the germline: Case 1: SDHC c...
  86. Dowaidar M, Abdelhamid H, Hällbrink M, Freimann K, Kurrikoff K, Zou X, et al. Magnetic Nanoparticle Assisted Self-assembly of Cell Penetrating Peptides-Oligonucleotides Complexes for Gene Delivery. Sci Rep. 2017;7:9159 pubmed publisher
    ..denoted PF220), PF221, PF222, PF223, PF224 and PF14) and three types of gene therapeutic agents (plasmid (pGL3), splicing correcting oligonucleotides (SCO), and small interfering RNA (siRNA) were investigated...
  87. Hirawake H, Taniwaki M, Tamura A, Kojima S, Kita K. Cytochrome b in human complex II (succinate-ubiquinone oxidoreductase): cDNA cloning of the components in liver mitochondria and chromosome assignment of the genes for the large (SDHC) and small (SDHD) subunits to 1q21 and 11q23. Cytogenet Cell Genet. 1997;79:132-8 pubmed
    ..In this study, the amino acid sequences of the large (cybL) and small (cybS) subunits of cytochrome b in human liver complex II were deduced from cDNAs isolated by homology ..
  88. Eng C, Kiuru M, Fernandez M, Aaltonen L. A role for mitochondrial enzymes in inherited neoplasia and beyond. Nat Rev Cancer. 2003;3:193-202 pubmed
    ..Understanding this link between inherited cancer syndromes and neurological disease could provide further insights into the mechanisms by which mitochondrial deficiencies lead to tumour development. ..
  89. Boedeker C, Neumann H, Maier W, Bausch B, Schipper J, Ridder G. Malignant head and neck paragangliomas in SDHB mutation carriers. Otolaryngol Head Neck Surg. 2007;137:126-9 pubmed
    ..PGL 1 is associated with mutations of the succinate dehydrogenase subunit D (SDHD) gene, PGL 3 is caused by SDHC gene mutations, and PGL 4 is caused by SDHB gene mutations...
  90. McWhinney S, Pasini B, Stratakis C. Familial gastrointestinal stromal tumors and germ-line mutations. N Engl J Med. 2007;357:1054-6 pubmed
  91. Pigny P, Cardot Bauters C, Do Cao C, Vantyghem M, Carnaille B, Pattou F, et al. Should genetic testing be performed in each patient with sporadic pheochromocytoma at presentation?. Eur J Endocrinol. 2009;160:227-31 pubmed publisher
    ..A germline mutation in one of the five susceptibility genes (VHL, RET, SDHD, SDHC, SDHB) was identified in eight patients (8%) with an age of onset between 13 and 57 years...