Transmission of cell stress from endoplasmic reticulum to mitochondria: enhanced expression of Lon protease.
AuthorsHori O, Ichinoda F, Tamatani T, Yamaguchi A, Sato N, Ozawa K, Kitao Y, Miyazaki M, Harding HP, Ron D, Tohyama M, M Stern D, Ogawa S
JournalJ Cell Biol
PubMed ID12082077
'The rat homologue of a mitochondrial ATP-dependent protease Lon was cloned from cultured astrocytes exposed to hypoxia. Expression of Lon was enhanced in vitro by hypoxia or ER stress, and in vivo by brain ischemia. These observations suggested that changes in nuclear gene expression (Lon) triggered by ER stress had ... More
Cytochrome c oxidase-deficient patients have distinct subunit assembly profiles.
AuthorsHanson BJ, Carrozzo R, Piemonte F, Tessa A, Robinson BH, Capaldi RA
JournalJ Biol Chem
PubMed ID11278850
'Cytochrome c oxidase (COX) deficiency is the most common respiratory chain defect in childhood and is clinically heterogeneous. We report a study of six patients with COX deficiencies. Two of the patients had as yet undefined defects, three patients had Surf-1 mutations, and one patient had a 15-base pair deletion ... More
Functional constraints of nuclear-mitochondrial DNA interactions in xenomitochondrial rodent cell lines.
AuthorsDey R, Barrientos A, Moraes CT
JournalJ Biol Chem
PubMed ID10908562
'The co-evolution of nuclear and mitochondrial genomes in vertebrates led to more than 100 specific interactions that are crucial for an optimized ATP generation. These interactions have been examined by introducing rat mtDNA into mouse cells devoid of mitochondrial DNA (mtDNA). When mtDNA-less cells derived from the common mouse (Mus ... More
Calcineurin regulates skeletal muscle metabolism via coordinated changes in gene expression.
AuthorsLong YC, Glund S, Garcia-Roves PM, Zierath JR
JournalJ Biol Chem
PubMed ID17107952
'The metabolic property of skeletal muscle adapts in response to an increased physiological demand by altering substrate utilization and gene expression. The calcium-regulated serine/threonine protein phosphatase calcineurin has been implicated in the transduction of motor neuron signals to alter gene expression programs in skeletal muscle. We utilized transgenic mice that ... More
Troglitazone-induced hepatic necrosis in an animal model of silent genetic mitochondrial abnormalities.
AuthorsOng MM, Latchoumycandane C, Boelsterli UA,
JournalToxicol Sci
PubMed ID17150972
Troglitazone, a first-generation thiazolidinedione antidiabetic drug, was withdrawn from the market due to an unacceptable risk of idiosyncratic hepatotoxicity. Troglitazone does not cause hepatotoxicity in normal healthy rodents, but it produces mitochondrial injury in vitro at high concentrations. The aim of this study was to explore whether genetic mitochondrial abnormalities ... More
Dysregulation of mitochondrial biogenesis in vascular endothelial and smooth muscle cells of aged rats.
AuthorsUngvari Z, Labinskyy N, Gupte S, Chander PN, Edwards JG, Csiszar A,
JournalAm J Physiol Heart Circ Physiol
PubMed ID18326800
Mitochondrial biogenesis is involved in the control of cell metabolism, signal transduction, and regulation of mitochondrial reactive oxygen species (ROS) production. Despite the central role of mitochondria in cellular aging and endothelial physiology, there are no studies extant investigating age-related alterations in mitochondrial biogenesis in blood vessels. Electronmicroscopy and confocal ... More
Resveratrol induces mitochondrial biogenesis in endothelial cells.
AuthorsCsiszar A, Labinskyy N, Pinto JT, Ballabh P, Zhang H, Losonczy G, Pearson K, de Cabo R, Pacher P, Zhang C, Ungvari Z,
JournalAm J Physiol Heart Circ Physiol
PubMed ID19429820
Pathways that regulate mitochondrial biogenesis are potential therapeutic targets for the amelioration of endothelial dysfunction and vascular disease. Resveratrol was shown to impact mitochondrial function in skeletal muscle and the liver, but its role in mitochondrial biogenesis in endothelial cells remains poorly defined. The present study determined whether resveratrol induces ... More
Expression of mtDNA and nDNA encoded respiratory chain proteins in chemically and genetically-derived Rho0 human fibroblasts: a comparison of subunit proteins in normal fibroblasts treated with ethidium bromide and fibroblasts from a patient with mtDNA depletion syndrome.
AuthorsMarusich MF, Robinson BH, Taanman JW, Kim SJ, Schillace R, Smith JL, Capaldi RA
JournalBiochim Biophys Acta
PubMed ID9540845
Although much progress has been made in identifying genetic defects associated with mitochondrial diseases, the protein expression patterns of most disorders are poorly understood. Here we use immunochemical techniques to describe subunit expression patterns of respiratory chain enzyme complexes II (succinate dehydrogenase: SD) and IV (cytochrome c oxidase: COX) in ... More
An immunocytochemical approach to detection of mitochondrial disorders.
Mitochondrial disorders can lead to a confusing array of symptoms, which frequently makes a diagnosis difficult. Traditional approaches to such diagnoses are based on enzyme activity assays, with further characterization provided by genetic analysis. However, these methods require relatively large sample sizes, are time-consuming, labor-intensive, and show variability between laboratories. ... More
Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin.
AuthorsMilakovic T, Johnson GV
JournalJ Biol Chem
PubMed ID15983033
There is significant evidence that energy production impairment and mitochondrial dysfunction play a role in the pathogenesis of Huntington disease. Nonetheless, the specific mitochondrial defects due to the presence of mutant huntingtin have not been fully elucidated. To determine the effects of mutant huntingtin on mitochondrial energy production, a thorough ... More