Cytopathies involving mitochondrial complex II
- PMID: 12231007
- DOI: 10.1016/s0098-2997(02)00012-2
Cytopathies involving mitochondrial complex II
Abstract
Complex II (succinate-ubiquinone oxidoreductase) is the smallest complex in the respiratory chain and contains four nuclear-encoded subunits SdhA, SdhB, SdhC, and SdhD. It functions both as a respiratory chain component and an essential enzyme of the TCA cycle. Electrons derived from succinate can thus be directly transferred to the ubiquinone pool. Major insights into the workings of complex II have been provided by crystal structures of closely related bacterial enzymes, which have also been genetically manipulated to answer questions of structure-function not approachable using the mammalian system. This information, together with that accrued over the years on bovine complex II and by recent advances in understanding in vivo synthesis of the non-heme iron co-factors of the enzyme, is allowing better recognition of improper functioning of human complex II in diseased states. The discussion in this review is thus limited to cytopathies arising because the enzyme itself is defective or depleted by lack of iron-sulfur clusters. There is a clear dichotomy of effects. Enzyme depletion and mutations in SDHA compromise TCA activity and energy production, whereas mutations in SDHB, SDHC, and SDHD induce paraganglioma. SDHC and SDHD are the first tumor suppressor genes of mitochondrial proteins.
Copyright 2002 Elsevier Science Ltd.
Similar articles
-
Phenotypic dichotomy in mitochondrial complex II genetic disorders.J Mol Med (Berl). 2001 Sep;79(9):495-503. doi: 10.1007/s001090100267. J Mol Med (Berl). 2001. PMID: 11692162 Review.
-
Two hydrophobic subunits are essential for the heme b ligation and functional assembly of complex II (succinate-ubiquinone oxidoreductase) from Escherichia coli.J Biol Chem. 1996 Jan 5;271(1):521-7. doi: 10.1074/jbc.271.1.521. J Biol Chem. 1996. PMID: 8550613
-
Localization of histidine residues responsible for heme axial ligation in cytochrome b556 of complex II (succinate:ubiquinone oxidoreductase) in Escherichia coli.Biochemistry. 1998 Mar 24;37(12):4148-59. doi: 10.1021/bi9716635. Biochemistry. 1998. PMID: 9521736
-
Retention of heme in axial ligand mutants of succinate-ubiquinone xxidoreductase (complex II) from Escherichia coli.J Biol Chem. 2001 Jun 1;276(22):18968-76. doi: 10.1074/jbc.M011270200. Epub 2001 Mar 19. J Biol Chem. 2001. PMID: 11259408
-
[Complex II (succinate-ubiquinone reductase) deficiency].Ryoikibetsu Shokogun Shirizu. 2001;(36):132-4. Ryoikibetsu Shokogun Shirizu. 2001. PMID: 11596343 Review. Japanese. No abstract available.
Cited by
-
Crystallographic investigation of the ubiquinone binding site of respiratory Complex II and its inhibitors.Biochim Biophys Acta Proteins Proteom. 2021 Sep;1869(9):140679. doi: 10.1016/j.bbapap.2021.140679. Epub 2021 Jun 3. Biochim Biophys Acta Proteins Proteom. 2021. PMID: 34089891 Free PMC article.
-
A spectrophotometric coupled enzyme assay to measure the activity of succinate dehydrogenase.Anal Biochem. 2013 Nov 1;442(1):19-23. doi: 10.1016/j.ab.2013.07.018. Epub 2013 Jul 22. Anal Biochem. 2013. PMID: 23886887 Free PMC article.
-
Whole-genome resequencing of Xishuangbanna fighting chicken to identify signatures of selection.Genet Sel Evol. 2016 Aug 26;48(1):62. doi: 10.1186/s12711-016-0239-4. Genet Sel Evol. 2016. PMID: 27565441 Free PMC article.
-
Crystallization of mitochondrial respiratory complex II from chicken heart: a membrane-protein complex diffracting to 2.0 A.Acta Crystallogr D Biol Crystallogr. 2005 Apr;61(Pt 4):380-7. doi: 10.1107/S0907444905000181. Epub 2005 Mar 24. Acta Crystallogr D Biol Crystallogr. 2005. PMID: 15805592 Free PMC article.
-
Electron transfer in Paracoccus denitrificans with the modified fbc operon.Folia Microbiol (Praha). 2009 Nov;54(6):475-82. doi: 10.1007/s12223-009-0067-9. Epub 2010 Feb 7. Folia Microbiol (Praha). 2009. PMID: 20140712
Publication types
MeSH terms
Substances
Grants and funding
LinkOut - more resources
Full Text Sources
Miscellaneous
