Sdhb

Summary

Gene Symbol: Sdhb
Description: succinate dehydrogenase complex, subunit B, iron sulfur (Ip)
Alias: 0710008N11Rik, succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial, iron-sulfur subunit of complex II, succinate dehydrogenase complex subunit B
Species: mouse
Products:     Sdhb

Top Publications

  1. Bjorkman J, Gould S, Crane D. Pex13, the mouse ortholog of the human peroxisome biogenesis disorder PEX13 gene: gene structure, tissue expression, and localization of the protein to peroxisomes. Genomics. 2002;79:162-8 pubmed
    ..We infer from these findings that targeted disruption of mouse Pex13 would provide an appropriate model for the study of PEX13 dysfunction in humans. ..
  2. 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
    ..We recently showed that SDHB mRNAs in hypoxic monocytes gain a stop codon mutation by APOBEC3A-mediated C-to-U RNA editing...
  3. Lussey Lepoutre C, Hollinshead K, Ludwig C, Menara M, Morin A, Castro Vega L, et al. Loss of succinate dehydrogenase activity results in dependency on pyruvate carboxylation for cellular anabolism. Nat Commun. 2015;6:8784 pubmed publisher
    ..Our results demonstrate that the loss of SDH reduces the metabolic plasticity of cells, suggesting vulnerabilities that can be targeted therapeutically. ..
  4. Cardaci S, Zheng L, Mackay G, van den Broek N, MacKenzie E, Nixon C, et al. Pyruvate carboxylation enables growth of SDH-deficient cells by supporting aspartate biosynthesis. Nat Cell Biol. 2015;17:1317-26 pubmed publisher
    ..Here, we generated Sdhb-ablated kidney mouse cells and used comparative metabolomics and stable-isotope-labelling approaches to identify ..
  5. Xekouki P, Szarek E, Bullova P, Giubellino A, Quezado M, Mastroyannis S, et al. Pituitary adenoma with paraganglioma/pheochromocytoma (3PAs) and succinate dehydrogenase defects in humans and mice. J Clin Endocrinol Metab. 2015;100:E710-9 pubmed publisher
    ..We also studied the pituitary gland and hormonal profile of Sdhb(+/-) mice and their wild-type littermates at different ages...
  6. Forner F, Kumar C, Luber C, Fromme T, Klingenspor M, Mann M. Proteome differences between brown and white fat mitochondria reveal specialized metabolic functions. Cell Metab. 2009;10:324-35 pubmed publisher
    ..In vivo comparison of organellar proteomes can thus directly address functional questions in metabolism. ..
  7. Yang H, Brosel S, Acin Perez R, Slavkovich V, Nishino I, Khan R, et al. Analysis of mouse models of cytochrome c oxidase deficiency owing to mutations in Sco2. Hum Mol Genet. 2010;19:170-80 pubmed publisher
    ..These mouse models should be of use in further studies of Sco2 function, as well as in testing therapeutic approaches to treat the human disorder. ..
  8. Xiao M, Yang H, Xu W, Ma S, Lin H, Zhu H, et al. Inhibition of ?-KG-dependent histone and DNA demethylases by fumarate and succinate that are accumulated in mutations of FH and SDH tumor suppressors. Genes Dev. 2012;26:1326-38 pubmed publisher
    ..These epigenetic alterations associated with mutations of FH and SDH likely contribute to tumorigenesis. ..
  9. Peralta S, Torraco A, Wenz T, Garcia S, Diaz F, Moraes C. Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage. Hum Mol Genet. 2014;23:1399-412 pubmed publisher
    ..These results showed that a partial defect in CI in neurons can lead to late-onset motor phenotypes without neuronal loss or oxidative damage. ..

More Information

Publications19

  1. Wiley S, Murphy A, Ross S, van der Geer P, Dixon J. MitoNEET is an iron-containing outer mitochondrial membrane protein that regulates oxidative capacity. Proc Natl Acad Sci U S A. 2007;104:5318-23 pubmed
    ..Cardiac mitochondria isolated from mitoNEET-null mice demonstrate a reduced oxidative capacity, suggesting that mito- NEET is an important iron-containing protein involved in the control of maximal mitochondrial respiratory rates. ..
  2. Mao P, Ardeshiri A, Jacks R, Yang S, Hurn P, Alkayed N. Mitochondrial mechanism of neuroprotection by CART. Eur J Neurosci. 2007;26:624-32 pubmed
    ..a potential direct interaction between CART and subunit B of the mitochondrial enzyme succinate dehydrogenase (SDHB)...
  3. Baumgart E, Vanhorebeek I, Grabenbauer M, Borgers M, Declercq P, Fahimi H, et al. Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse). Am J Pathol. 2001;159:1477-94 pubmed
  4. Diaz F, Thomas C, Garcia S, Hernandez D, Moraes C. Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency. Hum Mol Genet. 2005;14:2737-48 pubmed
    ..This COX10 KO mouse allowed us to correlate the muscle function with residual COX activity, an estimate that can help predict the progression pattern of human mitochondrial myopathies...
  5. Odegaard J, Lee M, Sogawa Y, Bertholet A, Locksley R, Weinberg D, et al. Perinatal Licensing of Thermogenesis by IL-33 and ST2. Cell. 2016;166:841-854 pubmed publisher
    ..Together, these data suggest that IL-33 and ST2 function as a developmental switch to license thermogenesis during the perinatal period. PAPERCLIP. ..
  6. Finley L, Haas W, Desquiret Dumas V, Wallace D, Procaccio V, Gygi S, et al. Succinate dehydrogenase is a direct target of sirtuin 3 deacetylase activity. PLoS ONE. 2011;6:e23295 pubmed publisher
    ..Two subunits of complex II (also known as succinate dehydrogenase, or SDH), SDHA and SDHB, interacted specifically with SIRT3...
  7. Letouze E, Martinelli C, Loriot C, Burnichon N, Abermil N, Ottolenghi C, et al. SDH mutations establish a hypermethylator phenotype in paraganglioma. Cancer Cell. 2013;23:739-52 pubmed publisher
    ..Epigenetic silencing was particularly severe in SDHB-mutated tumors, potentially explaining their malignancy...
  8. Ishihara T, Ban Ishihara R, Maeda M, Matsunaga Y, Ichimura A, Kyogoku S, et al. Dynamics of mitochondrial DNA nucleoids regulated by mitochondrial fission is essential for maintenance of homogeneously active mitochondria during neonatal heart development. Mol Cell Biol. 2015;35:211-23 pubmed publisher
    ..Thus, the dynamics of mtDNA nucleoids regulated by mitochondrial fission is required for neonatal cardiomyocyte development by promoting homogeneous distribution of active mitochondria throughout the cardiomyocytes. ..
  9. Lepoutre Lussey C, Thibault C, Buffet A, Morin A, Badoual C, Bénit P, et al. From Nf1 to Sdhb knockout: Successes and failures in the quest for animal models of pheochromocytoma. Mol Cell Endocrinol. 2016;421:40-8 pubmed publisher
    ..In this review, we present an overview of existing, successful or not, PPGL models, and a description of our own experience on the quest of Sdhb knockout mouse models of PPGL.
  10. Schilling B, Murray J, Yoo C, Row R, Cusack M, Capaldi R, et al. Proteomic analysis of succinate dehydrogenase and ubiquinol-cytochrome c reductase (Complex II and III) isolated by immunoprecipitation from bovine and mouse heart mitochondria. Biochim Biophys Acta. 2006;1762:213-22 pubmed
    ..The identification of several posttranslational modifications, including the covalent FAD modification of flavoprotein subunit 1 from Complex II, was possible due to high mass spectrometric sequence coverage. ..