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Gene Review

SDHB  -  succinate dehydrogenase complex, subunit B...

Homo sapiens

Synonyms: CWS2, IP, Ip, Iron-sulfur subunit of complex II, PGL4, ...
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Disease relevance of SDHB

  • Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma [1].
  • These findings extend the link between mitochondrial dysfunction and tumorigenesis and suggest that germline SDHB mutations are an important cause of pheochromocytoma susceptibility [1].
  • Early-onset renal cell carcinoma as a novel extraparaganglial component of SDHB-associated heritable paraganglioma [2].
  • The Mendelian tumour syndromes hereditary leiomyomatosis and renal cell cancer (HLRCC) and hereditary paragangliomatosis with phaeochromocytomas (HPGL) result from mutations in nuclear genes (FH and SDHB/C/D, respectively) that encode Krebs cycle enzymes [3].
  • Investigation of the role of SDHB inactivation in sporadic phaeochromocytoma and neuroblastoma [4].

Psychiatry related information on SDHB

  • Physical activity, assessed by accelerometer, was associated with average fiber SDH/GPDH (r = 0.78, P = 0.008) [5].

High impact information on SDHB

  • Complex II is comprised of two hydrophilic proteins, flavoprotein (Fp) and iron-sulfur protein (Ip), and two transmembrane proteins (CybL and CybS), as well as prosthetic groups required for electron transfer from succinate to ubiquinone [6].
  • We identified 11 hotspot loci of LOH/AI in the BRCA1/2 stroma, encompassing genes such as POLD1, which functions in DNA replication, and SDHB [7].
  • Hereditary paraganglioma syndrome has recently been shown to be caused by germline heterozygous mutations in three (SDHB, SDHC, and SDHD) of the four genes that encode mitochondrial succinate dehydrogenase [2].
  • The nuclear-encoded Krebs cycle enzymes, fumarate hydratase (FH) and succinate dehydrogenase (SDHB, -C and -D), act as tumour suppressors [8].
  • Recent studies suggest that germline SDHD and SDHB mutations are an important cause of familial and isolated phaeochromocytoma [9].

Chemical compound and disease context of SDHB

  • Although promoter region methylation was also detected in two neuroblastoma cell lines, this was not associated with silencing of SDHB expression, and treatment with a demethylating agent (5-azacytidine) did not increase SDH activity [4].
  • Two membrane enzymes, d-fructose dehydrogenase from Gluconobacter sp. (FDH) and sarcosine dehydrogenase from Pseudomonas putida (SDH), were for the first time immobilized onto the bilayer membranes of these type of vesicles; and the catalytic activity and enzymatic stability were measured and compared with the enzymes in a vesicle-free solution [10].
  • The present study in rats shows that cholestyramine given 4 and 24 h after paracetamol provided protection against both hepato- and nephrotoxicity; this was evidenced by reduced increments in plasma enzyme activities (SDH, GPT), indicating liver damage, and diminished retention of plasma and creatinine, indicating renal failure [11].
  • From myocardial biopsies, obtained before and after ischemia, complete electron transport system (ETS) enzyme activities (NDH, SDH, NCCR, SCCR, and COX) and lactate content were analyzed [12].

Biological context of SDHB


Anatomical context of SDHB

  • Germ-line mutations of the SDH genes have been found to account for nearly 10% of apparently sporadic cases [15].
  • Whereas SDH might be involved in hypoxic proliferation of paraganglia, FH might play an important role in the regulation of ammonium metabolism in smooth muscle cells [16].
  • These findings suggest that although germline SDHB mutations are an important cause of phaeochromocytoma susceptibility, somatic inactivation of SDHB does not have a major role in sporadic neural crest tumours and SDHB is not the target of 1p36 allele loss in neuroblastoma and phaeochromocytoma [4].
  • The novel SDHB p.R242C mutation was identified in a sporadic monolateral carotid body tumor [17].
  • In this study, we examined the level of expression of mRNAs encoding SDHB, SDHC, and SDHD in pheochromocytoma, pheochromocytoma subgroups, and normal adrenal gland, and compared the expression of these genes to the level of expression of related genes in the same tissues [18].

Associations of SDHB with chemical compounds

  • The SDHB mutation was a novel 3 base pair, in-frame deletion of AGC at nucleotide 583-585 encoding serine (delS195) [19].
  • Exchanging the region between the second and third cysteine clusters making up the [4Fe-4S] and [3Fe-4S] centers enabled transformants to grow on nonfermentable carbon sources, yet no SDH activity was observed in vitro [20].
  • Surprisingly, measurement of the activities of the polyol-pathway enzymes showed no difference in the levels of either AR or SDH between accumulators and nonaccumulators, even when the conversion of galactose to galactitol was used to measure AR activity in intact cells independently of SDH [21].
  • 3 alpha-Hydroxysteroid dehydrogenase (SDH) quantifies ursodeoxycholic acid, 3 alpha,12 alpha-diol-7-one-5 beta-cholanoic acid, 3 alpha-ol-7-one-5 beta-cholanoic acid, and 3 alpha,7 alpha-diol-12-one-5 beta-cholanoic acid in a manner similar to the more commonly measured bile acids, exemplified by taurocholic acid [22].
  • Parental SDH and WH strains exhibited comparable sensitivity to the PPI-disruptive effects of phencyclidine [23].

Other interactions of SDHB


Analytical, diagnostic and therapeutic context of SDHB

  • Gross SDHB deletions in patients with paraganglioma detected by multiplex PCR: a possible hot spot [15]?
  • Secondly, although the differential diagnosis between malignant and benign tumors remains a challenge, the risk of malignancy well exceeds the classical 10% in patients with extra-adrenal disease, and/or carriers of germ-line SDHB mutations [27].
  • SDHB mutation analysis was performed by denaturing high performance liquid chromatography followed by direct sequencing of aberrant PCR products [26].
  • In the majority of the tumours (approximately 90%), the enzyme-histochemical SDH reaction was negative and immunohistochemistry of catalytic subunits of complex II showed reduced expression of iron protein and enhanced expression of flavoprotein [28].
  • The London Daily/Nocturnal Hemodialysis Study was the first attempt to obtain data of SDH and NH that may be compared with conventional thrice weekly HD (CH) [29].


  1. Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. Astuti, D., Latif, F., Dallol, A., Dahia, P.L., Douglas, F., George, E., Sköldberg, F., Husebye, E.S., Eng, C., Maher, E.R. Am. J. Hum. Genet. (2001) [Pubmed]
  2. Early-onset renal cell carcinoma as a novel extraparaganglial component of SDHB-associated heritable paraganglioma. Vanharanta, S., Buchta, M., McWhinney, S.R., Virta, S.K., Peçzkowska, M., Morrison, C.D., Lehtonen, R., Januszewicz, A., Järvinen, H., Juhola, M., Mecklin, J.P., Pukkala, E., Herva, R., Kiuru, M., Nupponen, N.N., Aaltonen, L.A., Neumann, H.P., Eng, C. Am. J. Hum. Genet. (2004) [Pubmed]
  3. Evidence of increased microvessel density and activation of the hypoxia pathway in tumours from the hereditary leiomyomatosis and renal cell cancer syndrome. Pollard, P., Wortham, N., Barclay, E., Alam, A., Elia, G., Manek, S., Poulsom, R., Tomlinson, I. J. Pathol. (2005) [Pubmed]
  4. Investigation of the role of SDHB inactivation in sporadic phaeochromocytoma and neuroblastoma. Astuti, D., Morris, M., Krona, C., Abel, F., Gentle, D., Martinsson, T., Kogner, P., Neumann, H.P., Voutilainen, R., Eng, C., Rustin, P., Latif, F., Maher, E.R. Br. J. Cancer (2004) [Pubmed]
  5. Strength, skeletal muscle composition, and enzyme activity in multiple sclerosis. Kent-Braun, J.A., Ng, A.V., Castro, M., Weiner, M.W., Gelinas, D., Dudley, G.A., Miller, R.G. J. Appl. Physiol. (1997) [Pubmed]
  6. Crystal structure of mitochondrial respiratory membrane protein complex II. Sun, F., Huo, X., Zhai, Y., Wang, A., Xu, J., Su, D., Bartlam, M., Rao, Z. Cell (2005) [Pubmed]
  7. Total-genome analysis of BRCA1/2-related invasive carcinomas of the breast identifies tumor stroma as potential landscaper for neoplastic initiation. Weber, F., Shen, L., Fukino, K., Patocs, A., Mutter, G.L., Caldes, T., Eng, C. Am. J. Hum. Genet. (2006) [Pubmed]
  8. Accumulation of Krebs cycle intermediates and over-expression of HIF1alpha in tumours which result from germline FH and SDH mutations. Pollard, P.J., Brière, J.J., Alam, N.A., Barwell, J., Barclay, E., Wortham, N.C., Hunt, T., Mitchell, M., Olpin, S., Moat, S.J., Hargreaves, I.P., Heales, S.J., Chung, Y.L., Griffiths, J.R., Dalgleish, A., McGrath, J.A., Gleeson, M.J., Hodgson, S.V., Poulsom, R., Rustin, P., Tomlinson, I.P. Hum. Mol. Genet. (2005) [Pubmed]
  9. The pressure rises: update on the genetics of phaeochromocytoma. Maher, E.R., Eng, C. Hum. Mol. Genet. (2002) [Pubmed]
  10. Enzymatic activity and stability of D-fructose dehydrogenase and sarcosine dehydrogenase immobilized onto giant vesicles. Kato, K., Walde, P., Mitsui, H., Higashi, N. Biotechnol. Bioeng. (2003) [Pubmed]
  11. Cholestyramine as an antidote against paracetamol-induced hepato- and nephrotoxicity in the rat. Siegers, C.P., Möller-Hartmann, W. Toxicol. Lett. (1989) [Pubmed]
  12. Effect of partial oxygen supply on mitochondrial electron transport system during complete cardiac ischemia. Konuralp, C., Güner, S., Cakatay, U., Konuralp, Z., Yapící, N., Maçika, H., Aydoğan, H., Aykut-Aka, S., Alhan, C., Gültepe, M., Eren, E.E. Journal of cardiac surgery. (1999) [Pubmed]
  13. Familial malignant catecholamine-secreting paraganglioma with prolonged survival associated with mutation in the succinate dehydrogenase B gene. Young, A.L., Baysal, B.E., Deb, A., Young, W.F. J. Clin. Endocrinol. Metab. (2002) [Pubmed]
  14. No mutations but an increased frequency of SDHx polymorphisms in patients with sporadic and familial medullary thyroid carcinoma. Montani, M., Schmitt, A.M., Schmid, S., Locher, T., Saremaslani, P., Heitz, P.U., Komminoth, P., Perren, A. Endocr. Relat. Cancer (2005) [Pubmed]
  15. Gross SDHB deletions in patients with paraganglioma detected by multiplex PCR: a possible hot spot? Cascón, A., Montero-Conde, C., Ruiz-Llorente, S., Mercadillo, F., Letón, R., Rodríguez-Antona, C., Martínez-Delgado, B., Delgado, M., Díez, A., Rovira, A., Díaz, J.A., Robledo, M. Genes Chromosomes Cancer (2006) [Pubmed]
  16. On the association of succinate dehydrogenase mutations with hereditary paraganglioma. Baysal, B.E. Trends Endocrinol. Metab. (2003) [Pubmed]
  17. Paraganglioma Syndrome: SDHB, SDHC, and SDHD Mutations in Head and Neck Paragangliomas. Schiavi, F., Savvoukidis, T., Trabalzini, F., Grego, F., Piazza, M., Amist??, P., Dematt??, S., Piano, A.D., Cecchini, M.E., Erlic, Z., DE Lazzari, P., Mantero, F., Opocher, G. Ann. N. Y. Acad. Sci. (2006) [Pubmed]
  18. Expression of mRNAs for Succinate Dehydrogenase Subunits and Related Genes in Pheochromocytoma. Isobe, K., Nissato, S., Tatsuno, I., Yashiro, T., Takekoshi, K., Kawakami, Y. Ann. N. Y. Acad. Sci. (2006) [Pubmed]
  19. SDHB, SDHC, and SDHD mutation screen in sporadic and familial head and neck paragangliomas. Mhatre, A.N., Li, Y., Feng, L., Gasperin, A., Lalwani, A.K. Clin. Genet. (2004) [Pubmed]
  20. Studies on the assembly of complex II in yeast mitochondria using chimeric human/yeast genes for the iron-sulfur protein subunit. Saghbini, M., Broomfield, P.L., Scheffler, I.E. Biochemistry (1994) [Pubmed]
  21. Variation in sorbitol accumulation and polyol-pathway activity in cultured human proximal tubule cells. Flath, M.C., Bylander, J.E., Sens, D.A. Diabetes (1992) [Pubmed]
  22. Evaluation of the 3 alpha-hydroxysteroid dehydrogenase assay for ursodeoxycholic acid, and 7 oxo- and 12 oxo- bile acids. Haeffner, L.J., Gordon, S.J., Magen, J.S., Kowlessar, O.D. J. Lipid Res. (1980) [Pubmed]
  23. Sensitivity to sensorimotor gating-disruptive effects of apomorphine in two outbred parental rat strains and their F1 and N2 progeny. Swerdlow, N.R., Platten, A., Hanlon, F.M., Martinez, Z.A., Printz, M.P., Auerbach, P. Neuropsychopharmacology (2003) [Pubmed]
  24. Characterization of the human SDHC gene encoding of the integral membrane proteins of succinate-quinone oxidoreductase in mitochondria. Elbehti-Green, A., Au, H.C., Mascarello, J.T., Ream-Robinson, D., Scheffler, I.E. Gene (1998) [Pubmed]
  25. The genetic basis of pheochromocytoma. Gimm, O., Koch, C.A., Januszewicz, A., Opocher, G., Neumann, H.P. Frontiers of hormone research. (2004) [Pubmed]
  26. Molecular genetic analysis of FIH-1, FH, and SDHB candidate tumour suppressor genes in renal cell carcinoma. Morris, M.R., Maina, E., Morgan, N.V., Gentle, D., Astuti, D., Moch, H., Kishida, T., Yao, M., Schraml, P., Richards, F.M., Latif, F., Maher, E.R. J. Clin. Pathol. (2004) [Pubmed]
  27. Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor. Elder, E.E., Elder, G., Larsson, C. Journal of surgical oncology. (2005) [Pubmed]
  28. SDHD mutations in head and neck paragangliomas result in destabilization of complex II in the mitochondrial respiratory chain with loss of enzymatic activity and abnormal mitochondrial morphology. Douwes Dekker, P.B., Hogendoorn, P.C., Kuipers-Dijkshoorn, N., Prins, F.A., van Duinen, S.G., Taschner, P.E., van der Mey, A.G., Cornelisse, C.J. J. Pathol. (2003) [Pubmed]
  29. Nocturnal but not short hours quotidian hemodialysis requires an elevated dialysate calcium concentration. Al-Hejaili, F., Kortas, C., Leitch, R., Heidenheim, A.P., Clement, L., Nesrallah, G., Lindsay, R.M. J. Am. Soc. Nephrol. (2003) [Pubmed]
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