Human cytochrome c oxidase: structure, function, and deficiency.

Abstract:

:As the terminal component of the mitochondrial respiratory chain, cytochrome c oxidase plays a vital role in cellular energy transformation. Human cytochrome c oxidase is composed of 13 subunits. The three major subunits form the catalytic core and are encoded by mitochondrial DNA (mtDNA). The remaining subunits are nuclear-encoded. The primary sequence is known for all human subunits and the crystal structure of bovine heart cytochrome c oxidase has recently been reported. However, despite this wealth of structural information, the role of the nuclear encoded subunits is still poorly understood. Yeast cytochrome c oxidase is a close model of its human counterpart and provides a means of studying the effects of mutations on the assembly, structure, stability and function of the enzyme complex. Defects in cytochrome c oxidase function are found in a clinically heterogeneous group of disorders. The molecular defects that underlie these diseases may arise from mutations of either mitochondrial or the nuclear genomes or both. A significant number of cytochrome c oxidase deficiencies, often associated with other respiratory chain enzyme defects, are attributed to mutations of mtDNA. Mutations of mtDNA appear, nonetheless, uncommon in early childhood. Pedigree analysis and cell fusion experiments have demonstrated a nuclear involvement in some infantile cases but a specific genomic lesion has not yet been reported. Detailed analyses of the many steps involved in the biogenesis of cytochrome c oxidase, often pioneered in yeast, offer several starting points for further molecular characterizations of cytochrome c oxidase deficiencies observed in clinical practice.

journal_name

J Bioenerg Biomembr

authors

Taanman JW

doi

10.1023/a:1022638013825

subject

Has Abstract

pub_date

1997-04-01 00:00:00

pages

151-63

issue

2

eissn

0145-479X

issn

1573-6881

journal_volume

29

pub_type

杂志文章,评审
  • Size and selectivity of gap junction channels formed from different connexins.

    abstract::Gap junction channels have long been viewed as static structures containing a large-diameter, aqueous pore. This pore has a high permeability to hydrophilic molecules of approximately 900 daltons in molecular weight and a weak ionic selectivity. The evidence leading to these conclusions is reviewed in the context of m...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF02110109

    authors: Veenstra RD

    更新日期:1996-08-01 00:00:00

  • An integrated molecular modeling approach for the tryptase monomer-curcuminoid recognition analysis: conformational and bioenergetic features.

    abstract::Human mast cell tryptase has been shown as an activating enzyme in matrix degradation process. The previous study suggest that tryptase either alone or in joining with activation of metalloproteinases, can associate in extra cellular matrix damage and the possible destruction of the basement membrane resulting in phot...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-018-9777-5

    authors: Wongrattanakamon P,Ampasavate C,Sirithunyalug B,Jiranusornkul S

    更新日期:2018-12-01 00:00:00

  • Anion channels of the inner membrane of mammalian and yeast mitochondria.

    abstract::The inner membrane of yeast and mammalian mitochondria has been studied in situ with a patch clamp electrode. Anion channels were found in both cases, although their behavior and regulation are different. In mammalian mitochondria, the principal channel is of around 100 pS conductance and opens mainly under depolarize...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF02110642

    authors: Ballarin C,Sorgato MC

    更新日期:1996-04-01 00:00:00

  • New genes encoding subunits of a cytochrome bc1-analogous complex in the respiratory chain of the hyperthermoacidophilic crenarchaeon Sulfolobus acidocaldarius.

    abstract::The soxL gene from Sulfolobus acidocaldarius (DSM 639) encodes a Rieske iron-sulfur protein. In this study we report the identification of two open reading frames in its downstream region. The first one, named soxN, codes for a membrane protein bearing a resemblance to the b-type cytochromes of the cytochrome bc1 and ...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1023/a:1023742002493

    authors: Hiller A,Henninger T,Schäfer G,Schmidt CL

    更新日期:2003-04-01 00:00:00

  • Cox2A/Cox2B subunit interaction in Polytomella sp. cytochrome c oxidase: role of the Cox2B subunit extension.

    abstract::Subunit II of cytochrome c oxidase (Cox2) is usually encoded in the mitochondrial genome, synthesized in the organelle, inserted co-translationally into the inner mitochondrial membrane, and assembled into the respiratory complex. In chlorophycean algae however, the cox2 gene was split into the cox2a and cox2b genes, ...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-017-9728-6

    authors: Jiménez-Suárez A,Vázquez-Acevedo M,Miranda-Astudillo H,González-Halphen D

    更新日期:2017-12-01 00:00:00

  • Biological electron transfer.

    abstract::Many oxidoreductases are constructed from (a) local sites of strongly coupled substrate-redox cofactor partners participating in exchange of electron pairs, (b) electron pair/single electron transducing redox centers, and (c) nonadiabatic, long-distance, single-electron tunneling between weakly coupled redox centers. ...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF02110096

    authors: Moser CC,Page CC,Farid R,Dutton PL

    更新日期:1995-06-01 00:00:00

  • Simple oxygraphic analysis for the presence of adenylate kinase 1 and 2 in normal and tumor cells.

    abstract::The adenylate kinase (AK) isoforms network plays an important role in the intracellular energy transfer processes, the maintenance of energy homeostasis, and it is a major player in AMP metabolic signaling circuits in some highly-differentiated cells. For this purpose, a rapid and sensitive method was developed that e...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-016-9687-3

    authors: Klepinin A,Ounpuu L,Guzun R,Chekulayev V,Timohhina N,Tepp K,Shevchuk I,Schlattner U,Kaambre T

    更新日期:2016-10-01 00:00:00

  • Maturation of a eukaryotic cytochrome c in the cytoplasm of Escherichia coli without the assistance by a dedicated biogenesis apparatus.

    abstract::Maturation of c-type cytochromes involves the covalent and stereospecific enzymatic attachment of a heme b via thioether linkages to two conserved cysteines within apocytochromes. Horse cytochrome c is readily matured into its native holoform in the cytoplasm of E. coli when co-expressed with yeast cytochrome c heme l...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-010-9276-9

    authors: Tenger K,Khoroshyy P,Rákhely G,Zimányi L

    更新日期:2010-04-01 00:00:00

  • The road to the crystal structure of the cytochrome bc1 complex from the anoxigenic, photosynthetic bacterium Rhodobacter sphaeroides.

    abstract::The advantages of using bacterial systems to study the mechanism and function of cytochrome bc (1) complexes do not extend readily to their structural investigations. High quality crystals of bacterial complexes have been difficult to obtain despite the enzymes' smaller sizes and simpler subunit compositions compared ...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-008-9180-8

    authors: Xia D,Esser L,Elberry M,Zhou F,Yu L,Yu CA

    更新日期:2008-10-01 00:00:00

  • Inclusions in aged mitochondria.

    abstract::The formation of paracrystalline inclusions in mitochondria following postmortem aging in situ is further investigated. Inclusions are found to localize either in the mitochondrial intracristal space or between the inner and outer membrane. The greater majority of the inclusions are of the intracristal type. pH is not...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/BF00743275

    authors: Cheah KS,Cheah AM

    更新日期:1977-04-01 00:00:00

  • Partial characterization of the plasma membrane ATPase from a rho0 petite strain of Saccharomyces cerevisiae.

    abstract::Crude membrane preparations of a rho0 mutant of Saccharomyces cerevisiae exhibit Mg2+-dependent ATPase activity. Over the optimal pH range, 5.0-6.75, the apparent Vmax of the enzyme equals 590 nmoles of ATP hydrolyzed per minute per milligram protein, with an apparent Km for ATP of 1.3 mM. ATP hydrolysis is insensitiv...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/BF00744687

    authors: McDonough JP,Jaynes PK,Mahler HR

    更新日期:1980-08-01 00:00:00

  • Transplasma membrane electron transport in plants.

    abstract::The presence of transplasma membrane electron transport in a variety of plant cells and tissues is reported. It is now agreed that this property of eukaryotic cells is of ubiquitous nature. Studies with highly purified plasma membranes have established the presence of electron transport enzymes. Two types of activitie...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF00771013

    authors: Misra PC

    更新日期:1991-06-01 00:00:00

  • Proton transport-coupled unisite catalysis by the H(+)-ATPase from chloroplasts.

    abstract::Proton transport-coupled unisite catalysis was measured with the H(+)-ATPase from chloroplasts. The reaction was measured in the ATP hydrolysis direction under deenergized conditions and in the ATP synthesis direction under energized conditions. The equilibrium constant of the enzyme does not change upon energization,...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF00762367

    authors: Gräber P,Labahn A

    更新日期:1992-10-01 00:00:00

  • The membrane-bound electron transport system of Methanosarcina species.

    abstract::Members of the genus Methanosarcina are strictly anaerobic archaea that derive their metabolic energy from the conversion of a restricted number of substrates to methane. H2 + CO2 are converted to CH4 via the CO2-reducing pathway, while methanol and methylamines are metabolized by the methylotrophic pathway. Two novel...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1023/b:jobb.0000019598.64642.97

    authors: Deppenmeier U

    更新日期:2004-02-01 00:00:00

  • Mitochondria and FOXO3 in stem cell homeostasis, a window into hematopoietic stem cell fate determination.

    abstract::The production of all blood cells from hematopoietic stem cells (HSC) is highly sensitive to reactive oxygen species (ROS). Cumulating evidence suggests that mitochondria are critical for HSC fate determination. FOXO are known regulators of anti-oxidant response and key to the maintenance of HSC. Recent works indicate...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/s10863-017-9719-7

    authors: Liang R,Ghaffari S

    更新日期:2017-08-01 00:00:00

  • Soy isoflavones and cellular mechanics.

    abstract::Soy isoflavones are diphenolic compounds that are frequently used for alternative treatment of ageing symptoms in both genders. They operate at principally two hierarchical levels of functional organization - cellular and molecular, while these 'types' of action appear to have indefinite borders. Soy isoflavone action...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/s10863-013-9536-6

    authors: Ajdžanović VZ,Medigović IM,Pantelić JB,Milošević VLj

    更新日期:2014-04-01 00:00:00

  • The Saccharomyces cerevisiae ATP synthase.

    abstract::The ATP synthase of the yeast Saccharomyces cerevisiae is composed of 20 different subunits whose primary structure is known. The organization of proteins that constitute the membranous domain is now under investigation. Cysteine insertions combined with the use of nonpermeant maleimide reagents and cross-linking reag...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1023/a:1005580020547

    authors: Velours J,Arselin G

    更新日期:2000-08-01 00:00:00

  • The sulfonylurea-inhibited NADH oxidase activity of HeLa cell plasma membranes has properties of a protein disulfide-thiol oxidoreductase with protein disulfide-thiol interchange activity.

    abstract::Plasma membrane vesicles of HeLa cells are characterized by a drug-responsive oxidation of NADH. The NADH oxidation takes place in an argon or nitrogen atmosphere and in samples purged of oxygen. Direct assay of protein thiols by reaction with 5,5'-dithiobis-(2-nitrobenzoic acid) (DTNB; Ellman's reagent), suggests tha...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1023/a:1020594214379

    authors: Morré DJ,Chueh PJ,Lawler J,Morré DM

    更新日期:1998-10-01 00:00:00

  • Mutations in the Atp1p and Atp3p subunits of yeast ATP synthase differentially affect respiration and fermentation in Saccharomyces cerevisiae.

    abstract::ATP1-111, a suppressor of the slow-growth phenotype of yme1Delta lacking mitochondrial DNA is due to the substitution of phenylalanine for valine at position 111 of the alpha-subunit of mitochondrial ATP synthase (Atp1p in yeast). The suppressing activity of ATP1-111 requires intact beta (Atp2p) and gamma (Atp3p) subu...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-007-9071-4

    authors: Francis BR,White KH,Thorsness PE

    更新日期:2007-04-01 00:00:00

  • Does the gamma subunit move to an abortive position of ATP hydrolysis when the F1.ADP.Mg complex isomerizes to the inactive F1*.ADP.Mg complex?

    abstract::F1-ATPases transiently entrap inhibitory MgADP in a catalytic site during turnover when noncatalytic sites are not saturated with ATP. An initial burst of ATP hydrolysis rapidly decelerates to a slow intermediate rate that gradually accelerates to a final steady-state rate. Transition from the intermediate to the fina...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF02113985

    authors: Allison WS,Jault JM,Dou C,Grodsky NB

    更新日期:1996-10-01 00:00:00

  • The response of plant mitochondria to media of high solute content.

    abstract::The state-3 rate of respiration of potato tuber mitochondria is inhibited by concentrations of KCl or NaCl above 125 mM, and by concentrations of sucrose, lactose, or maltose above 500 mM, but not at all by mannitol, glucose, glycine, or proline up to a concentration of 1500 mM in the medium. Mitochondria from caulifl...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/BF00748958

    authors: Campbell LC,Raison JK,Brady CJ

    更新日期:1976-06-01 00:00:00

  • Effects of methylglyoxal and pyridoxamine in rat brain mitochondria bioenergetics and oxidative status.

    abstract::Advanced glycation end products (AGEs) and methylglyoxal (MG), an important intermediate in AGEs synthesis, are thought to contribute to protein aging and to the pathogenesis of age-and diabetes-associated complications. This study was intended to investigate brain mitochondria bioenergetics and oxidative status of ra...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-014-9551-2

    authors: Cardoso S,Carvalho C,Marinho R,Simões A,Sena CM,Matafome P,Santos MS,Seiça RM,Moreira PI

    更新日期:2014-10-01 00:00:00

  • The ferrochelatase gene structure and molecular defects associated with erythropoietic protoporphyria.

    abstract::Ferrochelatase [heme synthase, protoheme ferrolyase (EC 4.99.1.1)], the terminal enzyme of the heme biosynthetic pathway, catalyzes the incorporation of ferrous ion into protoporphyrin IX to form protoheme IX. The genes and cDNAs for ferrochelatase from mammals and micro-organisms have been isolated. The gene for huma...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF02110038

    authors: Taketani S,Fujita H

    更新日期:1995-04-01 00:00:00

  • TIPE2 suppresses progression and tumorigenesis of the oral tongue squamous cell carcinoma by regulating FoxP3+ regulatory T cells.

    abstract::To discover the effect of tumor necrosis factor-alpha-induced protein 8-like 2 (TIPE2) on the oral tongue squamous cell carcinoma (OTSCC) via affecting FoxP3+ regulatory T (Treg) cells. Immunohistochemistry was conducted to examine TIPE2 and FoxP3 expressions in OTSCC tumor tissues and corresponding oral mucosa. Tca81...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-020-09840-w

    authors: Zhao LL

    更新日期:2020-08-01 00:00:00

  • NYGGF4 (PID1) effects on insulin resistance are reversed by metformin in 3T3-L1 adipocytes.

    abstract::NYGGF4 (also called PID1) is a recently discovered gene that is involved in obesity-related insulin resistance (IR). We aimed in the present study to further elucidate the effects of NYGGF4 on IR and the underlying mechanisms through using metformin treatment in 3T3-L1 adipocytes. Our data showed that the metformin pr...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-012-9472-x

    authors: Qiu J,Wang YM,Shi CM,Yue HN,Qin ZY,Zhu GZ,Cao XG,Ji CB,Cui Y,Guo XR

    更新日期:2012-12-01 00:00:00

  • Nm23-H1/NDP kinase folding intermediates and cancer: a hypothesis.

    abstract::The Nm23-H1/nucleoside diphosphate (NDP) kinase A is a metastasis suppressor, besides its enzymatic activity. The mutant S120G has been found in high-grade neuroblastomas. The mutant protein, once denatured in urea, is unable to refold in vitro. A size-exclusion chromatography analysis of the folding/association pathw...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-006-9042-1

    authors: Lascu I

    更新日期:2006-08-01 00:00:00

  • Iron-induced oxidant stress leads to irreversible mitochondrial dysfunctions and fibrosis in the liver of chronic iron-dosed gerbils. The effect of silybin.

    abstract::Hepatic iron toxicity because of iron overload seems to be mediated by lipid peroxidation of biological membranes and the associated organelle dysfunctions. However, the basic mechanisms underlying this process in vivo are still little understood. Gerbils were dosed with weekly injections of iron-dextran alone or in c...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1023/a:1005512014280

    authors: Masini A,Ceccarelli D,Giovannini F,Montosi G,Garuti C,Pietrangelo A

    更新日期:2000-04-01 00:00:00

  • Fatty acid synthesis by Chlamydomonas reinhardtii in phosphorus limitation.

    abstract::The importance of crucial nutrient factors like Phosphate (P) and their limited availability leads to variable fluctuations in fatty acid and phospholipid synthesis in the green alga. These fatty acids and phospholipids are an imperative byproduct of alga which used in biofuel production. The production of phospholipi...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-019-09813-8

    authors: Qari HA,Oves M

    更新日期:2020-02-01 00:00:00

  • A unifying concept for ion translocation by retinal proteins.

    abstract::First, halorhodopsin is capable of pumping protons after illumination with green and blue light in the same direction as chloride. Second, mutated bacteriorhodopsin where the proton acceptor Asp85 and the proton donor Asp96 are replaced by Asn showed proton pump activity after illumination with blue light in the same ...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF00762676

    authors: Oesterhelt D,Tittor J,Bamberg E

    更新日期:1992-04-01 00:00:00

  • Mitochondrial ATP synthase: a bioinformatic approach reveals new insights about the roles of supernumerary subunits g and A6L.

    abstract::The mitochondrial ATP synthase is a membrane protein complex which couples the proton gradient across the mitochondrial inner membrane to the synthesis of ATP from ADP+Pi. The complex is composed of essential subunits for its motor functions and supernumerary subunits, the roles of which remain to be elucidated. Subun...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-004-8998-y

    authors: Hong S,Pedersen PL

    更新日期:2004-12-01 00:00:00