نتایج جستجو برای: mitochondrial enzymes

تعداد نتایج: 253035  

Journal: :Molecular cell 2013
Alexander S Hebert Kristin E Dittenhafer-Reed Wei Yu Derek J Bailey Ebru Selin Selen Melissa D Boersma Joshua J Carson Marco Tonelli Allison J Balloon Alan J Higbee Michael S Westphall David J Pagliarini Tomas A Prolla Fariba Assadi-Porter Sushmita Roy John M Denu Joshua J Coon

Calorie restriction (CR) extends life span in diverse species. Mitochondria play a key role in CR adaptation; however, the molecular details remain elusive. We developed and applied a quantitative mass spectrometry method to probe the liver mitochondrial acetyl-proteome during CR versus control diet in mice that were wild-type or lacked the protein deacetylase SIRT3. Quantification of 3,285 ace...

Journal: :DNA repair 2010
Matthew L Bochman Nasim Sabouri Virginia A Zakian

Helicases are ubiquitous enzymes found in all organisms that are necessary for all (or virtually all) aspects of nucleic acid metabolism. The Pif1 helicase family is a group of 5'-->3' directed, ATP-dependent, super family IB helicases found in nearly all eukaryotes. Here, we review the discovery, evolution, and what is currently known about these enzymes in Saccharomyces cerevisiae (ScPif1 and...

2014
Jenny D.Y. Chow Robert T. Lawrence Marin E. Healy John E. Dominy Jason A. Liao David S. Breen Frances L. Byrne Brandon M. Kenwood Carolin Lackner Saeko Okutsu Valeria R. Mas Stephen H. Caldwell Jose L. Tomsig Gregory J. Cooney Pere B. Puigserver Nigel Turner David E. James Judit Villén Kyle L. Hoehn

Lipid deposition in the liver is associated with metabolic disorders including fatty liver disease, type II diabetes, and hepatocellular cancer. The enzymes acetyl-CoA carboxylase 1 (ACC1) and ACC2 are powerful regulators of hepatic fat storage; therefore, their inhibition is expected to prevent the development of fatty liver. In this study we generated liver-specific ACC1 and ACC2 double knock...

2017
Franziska Foertsch Mykhailo Ilchenko Thomas Heitkamp Silke Nossmann Birgit Hoffmann Ilka Starke Ralf Mrowka Christoph Biskup Michael Borsch

Cytochrome C oxidase and FoF1-ATP synthase constitute complex IV and V, respectively, of the five membrane-bound enzymes in mitochondria comprising the respiratory chain. These enzymes are located in the inner mitochondrial membrane (IMM), which exhibits large invaginations called cristae. According to recent cryo-tomography, FoF1-ATP synthases are located predominantly at the rim of the crista...

Journal: :Journal of oral science 2017
Sumita Banerjee Saikat Mukherjee Sanjib Mitra Pallav Singhal

Reactive oxygen species, if produced in excess by oxidative phosphorylation, contributes to mitochondrial DNA damage and progressive respiratory chain dysfunction, leading to various diseases including carcinogenesis. Mitochondria are susceptible to oxidative stress (OS) owing to lack of introns, protective histones, and DNA repair enzymes. However, mitochondria are protected from OS by numerou...

Journal: :American journal of human genetics 2012
Beatriz Garcia-Diaz Mario H Barros Simone Sanna-Cherchi Valentina Emmanuele Hasan O Akman Claudia C Ferreiro-Barros Rita Horvath Saba Tadesse Nader El Gharaby Salvatore DiMauro Darryl C De Vivo Aly Shokr Michio Hirano Catarina M Quinzii

Defects of mitochondrial protein synthesis are clinically and genetically heterogeneous. We previously described a male infant who was born to consanguineous parents and who presented with severe congenital encephalopathy, peripheral neuropathy, myopathy, and lactic acidosis associated with deficiencies of multiple mitochondrial respiratory-chain enzymes and defective mitochondrial translation....

Journal: :The Journal of clinical investigation 1983
D S Loose P B Kan M A Hirst R A Marcus D Feldman

Ketoconazole has recently been shown to interfere with steroidogenesis in patients and rat in vitro systems. In this study we attempted to elucidate the site of inhibition in the adrenal gland. Although ketoconazole impaired adrenocorticotropic hormone stimulated cyclic (c)AMP production, dibutyrl cAMP addition did not bypass the steroidogenic blockade indicating that the critical ketoconazole-...

1998
Igor Morgunov Paul A. Srere

The interactions between pig heart citrate synthase and mitochondrial malate dehydrogenase or cytosolic malate dehydrogenase were studied using the frontal analysis method of gel filtration and by precipitation in polyethylene glycol. This method showed that an interaction between citrate synthase and mitochondrial malate dehydrogenase occurred but no interaction between citrate synthase and cy...

Journal: :The Biochemical journal 2011
Damon A Lowes Helen F Galley

Oxidative stress and mitochondrial dysfunction are common features in patients with sepsis and organ failure. Within mitochondria, superoxide is converted into hydrogen peroxide by MnSOD (manganese-containing superoxide dismutase), which is then detoxified by either the mGSH (mitochondrial glutathione) system, using the enzymes mGPx-1 (mitochondrial glutathione peroxidase-1), GRD (glutathione r...

Journal: :Brain : a journal of neurology 2005
Anna Krasnianski Marcus Deschauer Stephan Neudecker Frank N Gellerich Tobias Müller Benedikt G Schoser Michael Krasnianski Stephan Zierz

Previous findings suggested specific mitochondrial dysfunction in skeletal muscle of patients with amyotrophic lateral sclerosis (ALS). To answer the question of whether the dysfunction is specific, we investigated the histochemical distribution of mitochondrial marker activities, the ratio of mitochondrial (mt) versus nuclear (n) DNA, and the activities of citrate synthase (CS) and respiratory...

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