Mitochondrial Proteins

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  Subject Areas on Research

  • A high-fat diet elicits differential responses in genes coordinating oxidative metabolism in skeletal muscle of lean and obese individuals. 
  • A structural model of the alternative oxidase of plant mitochondria. 
  • A truncated progesterone receptor (PR-M) localizes to the mitochondrion and controls cellular respiration. 
  • Acetylation of mitochondrial proteins. 
  • Acquisition of mitochondrial dysregulation and resistance to mitochondrial-mediated apoptosis after genotoxic insult in normal human fibroblasts: a possible model for early stage carcinogenesis. 
  • Actions of steroids in mitochondria. 
  • Activation of mitophagy leads to decline in Mfn2 and loss of mitochondrial mass in Fuchs endothelial corneal dystrophy. 
  • Activation of the plant mitochondrial alternative oxidase: insights from site-directed mutagenesis. 
  • Acyl-CoA thioesterase-2 facilitates mitochondrial fatty acid oxidation in the liver. 
  • Adenovirus-mediated overexpression of uncoupling protein-2 in pancreatic islets of Zucker diabetic rats increases oxidative activity and improves beta-cell function. 
  • Adipose-specific deletion of TFAM increases mitochondrial oxidation and protects mice against obesity and insulin resistance. 
  • Adrenoceptors, uncoupling proteins, and energy expenditure. 
  • Analysis of microsatellite mutations in the mitochondrial DNA of Saccharomyces cerevisiae. 
  • Axonal neuropathy with optic atrophy is caused by mutations in mitofusin 2. 
  • Bacterial lipopolysaccharide induces uncoupling protein-2 expression in hepatocytes by a tumor necrosis factor-alpha-dependent mechanism. 
  • Bioenergetic defect associated with mKATP channel opening in a mouse model carrying a mitofusin 2 mutation. 
  • Branched-Chain Aminotransferases Control TORC1 Signaling in Saccharomyces cerevisiae. 
  • CERKL interacts with mitochondrial TRX2 and protects retinal cells from oxidative stress-induced apoptosis. 
  • CHCHD2 inhibits apoptosis by interacting with Bcl-x L to regulate Bax activation. 
  • Characterization of C1q receptor expression on human phagocytic cells: effects of PDBu and fMLP. 
  • Characterization of an iron-sulfur cluster assembly protein (ISU1) from Schizosaccharomyces pombe. 
  • Characterization of transformed Arabidopsis with altered alternative oxidase levels and analysis of effects on reactive oxygen species in tissue. 
  • Charcot-Marie-Tooth with pyramidal signs is genetically heterogeneous: families with and without MFN2 mutations. 
  • Clinical heterogeneity of mitochondrial NAD kinase deficiency caused by a NADK2 start loss variant. 
  • Comparison of proteomic and metabolomic profiles of mutants of the mitochondrial respiratory chain in Caenorhabditis elegans. 
  • Cross-linking and disulfide bond formation of introduced cysteine residues suggest a modified model for the tertiary structure of URF13 in the pore-forming oligomers. 
  • Cross-linking of the cms-T maize mitochondrial pore-forming protein URF13 by N,N'-dicyclohexylcarbodiimide and its effect on URF13 sensitivity to fungal toxins. 
  • Crystal structures of T. brucei MRP1/MRP2 guide-RNA binding complex reveal RNA matchmaking mechanism. 
  • Deficiency in SLC25A1, encoding the mitochondrial citrate carrier, causes combined D-2- and L-2-hydroxyglutaric aciduria. 
  • Diet-induced changes in uncoupling proteins in obesity-prone and obesity-resistant strains of mice. 
  • Diminished acyl-CoA synthetase isoform 4 activity in INS 832/13 cells reduces cellular epoxyeicosatrienoic acid levels and results in impaired glucose-stimulated insulin secretion. 
  • Disrupted signaling and inhibited regeneration in obese mice with fatty livers: implications for nonalcoholic fatty liver disease pathophysiology. 
  • Disruption of the Ang II type 1 receptor promotes longevity in mice. 
  • Disruption of the uncoupling protein-2 gene in mice reveals a role in immunity and reactive oxygen species production. 
  • Dysregulation of mitochondrial dynamics proteins are a targetable feature of human tumors. 
  • Dystonia with and without deafness is caused by TIMM8A mutation. 
  • Effects of burn injury, cold stress and cutaneous wound injury on the morphology and energy metabolism of murine brown adipose tissue (BAT) in vivo. 
  • Endotoxin-stimulated nitric oxide production inhibits expression of cytochrome c oxidase in ANA-1 murine macrophages. 
  • Energy metabolism in uncoupling protein 3 gene knockout mice. 
  • Erk2 phosphorylation of Drp1 promotes mitochondrial fission and MAPK-driven tumor growth. 
  • Erythropoietin activates mitochondrial biogenesis and couples red cell mass to mitochondrial mass in the heart. 
  • Expression of mitofusin 2(R94Q) in a transgenic mouse leads to Charcot-Marie-Tooth neuropathy type 2A. 
  • Fatty acid homeostasis and induction of lipid regulatory genes in skeletal muscles of peroxisome proliferator-activated receptor (PPAR) alpha knock-out mice. Evidence for compensatory regulation by PPAR delta. 
  • Features of programmed cell death in intact Xenopus oocytes and early embryos revealed by near-infrared fluorescence and real-time monitoring. 
  • Fungal toxins bind to the URF13 protein in maize mitochondria and Escherichia coli. 
  • Genomic characterization of POS5, the Saccharomyces cerevisiae mitochondrial NADH kinase. 
  • Glucose and weight control in mice with a designed ghrelin O-acyltransferase inhibitor. 
  • Glutamate excitotoxicity in neurons triggers mitochondrial and endoplasmic reticulum accumulation of Parkin, and, in the presence of N-acetyl cysteine, mitophagy. 
  • HDAC6 maintains mitochondrial connectivity under hypoxic stress by suppressing MARCH5/MITOL dependent MFN2 degradation. 
  • Homocysteine-mediated modulation of mitochondrial dynamics in retinal ganglion cells. 
  • Human pluripotent stem cells decouple respiration from energy production. 
  • Identifying single S-nitrosothiol sites with cardioprotection. 
  • In vivo cytochrome and alternative pathway respiration in leaves of Arabidopsis thaliana plants with altered alternative oxidase under different light conditions. 
  • Incongruence between primary sequence data and the distribution of a mitochondrial atp1 group II intron among ferns and horsetails. 
  • Increased mtDNA levels without change in mitochondrial enzymes in peripheral blood mononuclear cells of infants born to HIV-infected mothers on antiretroviral therapy. 
  • Individuals with mutations in XPNPEP3, which encodes a mitochondrial protein, develop a nephronophthisis-like nephropathy. 
  • Induction by leptin of uncoupling protein-2 and enzymes of fatty acid oxidation. 
  • Influence of ABCB1, ABCC1, ABCC2, and ABCG2 haplotypes on the cellular exposure of nelfinavir in vivo. 
  • Inherited variants in mitochondrial biogenesis genes may influence epithelial ovarian cancer risk. 
  • Interferon regulatory factor-1 regulates reconstituted extracellular matrix (rECM)-mediated apoptosis in human mammary epithelial cells. 
  • LETM1, a gene deleted in Wolf-Hirschhorn syndrome, encodes an evolutionarily conserved mitochondrial protein. 
  • Lack of association between autism and SLC25A12. 
  • Learning new tricks from old dogs: beta-adrenergic receptors teach new lessons on firing up adipose tissue metabolism. 
  • Lipids up-regulate uncoupling protein 2 expression in rat hepatocytes. 
  • Lipopolysaccharide induces oxidative cardiac mitochondrial damage and biogenesis. 
  • Loss of Function Mutations in NNT Are Associated With Left Ventricular Noncompaction. 
  • Loss of sirtuin 4 leads to elevated glucose- and leucine-stimulated insulin levels and accelerated age-induced insulin resistance in multiple murine genetic backgrounds. 
  • Low plasma leptin in response to dietary fat in diabetes- and obesity-prone mice. 
  • MFN2 mutation distribution and genotype/phenotype correlation in Charcot-Marie-Tooth type 2. 
  • Mammalian Sir2 homolog SIRT3 regulates global mitochondrial lysine acetylation. 
  • Manganese porphyrin reduces renal injury and mitochondrial damage during ischemia/reperfusion. 
  • Mitochondrial FtsZ in a chromophyte alga. 
  • Mitochondrial NADP(H) deficiency due to a mutation in NADK2 causes dienoyl-CoA reductase deficiency with hyperlysinemia. 
  • Mitochondrial acetylome analysis in a mouse model of alcohol-induced liver injury utilizing SIRT3 knockout mice. 
  • Mitochondrial adaptations to obesity-related oxidant stress. 
  • Mitochondrial protein acetylation regulates metabolism. 
  • Mitochondrial proteins that regulate apoptosis and necrosis are induced in mouse fatty liver. 
  • Mitochondrial sirtuins. 
  • Mitochondrial uncoupling: role of uncoupling protein anion carriers and relationship to thermogenesis and weight control "the benefits of losing control". 
  • Mitofusins 1/2 and ERRalpha expression are increased in human skeletal muscle after physical exercise. 
  • Mitophagy defects arising from BNip3 loss promote mammary tumor progression to metastasis. 
  • Molecular basis of disease susceptibility in the Texas cytoplasm of maize. 
  • Molecular genetics of autosomal-dominant axonal Charcot-Marie-Tooth disease. 
  • Muscle oxidative capacity during IL-6-dependent cancer cachexia. 
  • Mutation analysis of CHCHD2 gene in Chinese Han familial essential tremor patients and familial Parkinson's disease patients. 
  • Mutations in SCO2 are associated with autosomal-dominant high-grade myopia. 
  • Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A. 
  • Mutations in the novel mitochondrial protein REEP1 cause hereditary spastic paraplegia type 31. 
  • N,N'-dicyclohexylcarbodiimide cross-linking suggests a central core of helices II in oligomers of URF13, the pore-forming T-toxin receptor of cms-T maize mitochondria. 
  • NLRX1 Sequesters STING to Negatively Regulate the Interferon Response, Thereby Facilitating the Replication of HIV-1 and DNA Viruses. 
  • Nitric oxide synthase-2 regulates mitochondrial Hsp60 chaperone function during bacterial peritonitis in mice. 
  • Obesity induces expression of uncoupling protein-2 in hepatocytes and promotes liver ATP depletion. 
  • Oxygen flux analysis to understand the biological function of sirtuins. 
  • PD-linked CHCHD2 mutations impair CHCHD10 and MICOS complex leading to mitochondria dysfunction. 
  • PGC-1alpha mRNA expression is influenced by metabolic perturbation in exercising human skeletal muscle. 
  • PKA/AKAP1 and PP2A/Bβ2 regulate neuronal morphogenesis via Drp1 phosphorylation and mitochondrial bioenergetics. 
  • PTH/PTHrP Receptor Mediates Cachexia in Models of Kidney Failure and Cancer. 
  • Persistent nuclear factor-kappa B activation in Ucp2-/- mice leads to enhanced nitric oxide and inflammatory cytokine production. 
  • Persistent oxidative stress due to absence of uncoupling protein 2 associated with impaired pancreatic beta-cell function. 
  • Pharmacological targeting of the mitochondrial phosphatase PTPMT1. 
  • Phenotypic abnormalities in macrophages from leptin-deficient, obese mice. 
  • Polyunsaturated fatty acids stimulate hepatic UCP-2 expression via a PPARalpha-mediated pathway. 
  • Postlipopolysaccharide oxidative damage of mitochondrial DNA. 
  • Probiotics and antibodies to TNF inhibit inflammatory activity and improve nonalcoholic fatty liver disease. 
  • Progressive mitochondrial protein lysine acetylation and heart failure in a model of Friedreich's ataxia cardiomyopathy. 
  • Proteomic identification of a stress protein, mortalin/mthsp70/GRP75: relevance to Parkinson disease. 
  • Proteomic profiling of a layered tissue reveals unique glycolytic specializations of photoreceptor cells. 
  • Pyruvate dehydrogenase complex and nicotinamide nucleotide transhydrogenase constitute an energy-consuming redox circuit. 
  • RALA and RALBP1 regulate mitochondrial fission at mitosis. 
  • ROS signaling, oxidative stress and Nrf2 in pancreatic beta-cell function. 
  • RanBP2 modulates Cox11 and hexokinase I activities and haploinsufficiency of RanBP2 causes deficits in glucose metabolism. 
  • Regulated protein denitrosylation by cytosolic and mitochondrial thioredoxins. 
  • Regulation of alternative oxidase activity in six wild monocotyledonous species. An in vivo study at the whole root level. 
  • Regulation of alternative oxidase kinetics by pyruvate and intermolecular disulfide bond redox status in soybean seedling mitochondria. 
  • Regulation of mitochondrial morphology by APC/CCdh1-mediated control of Drp1 stability. 
  • Regulation of mitochondrial processes by protein S-nitrosylation. 
  • Regulation of plant alternative oxidase activity: a tale of two cysteines. 
  • Regulation of skeletal muscle oxidative capacity and insulin signaling by the mitochondrial rhomboid protease PARL. 
  • Regulation of the cyanide-resistant alternative oxidase of plant mitochondria. Identification of the cysteine residue involved in alpha-keto acid stimulation and intersubunit disulfide bond formation. 
  • Regulation of the uncoupling protein gene (Ucp) by beta 1, beta 2, and beta 3-adrenergic receptor subtypes in immortalized brown adipose cell lines. 
  • Regulation of the uncoupling protein-2 gene in INS-1 beta-cells by oleic acid. 
  • Resistance to cerebral ischemic injury in UCP2 knockout mice: evidence for a role of UCP2 as a regulator of mitochondrial glutathione levels. 
  • Restoration of the cystic fibrosis transmembrane conductance regulator function by splicing modulation. 
  • Role of alternative oxidase gene in pathogenesis of Cryptococcus neoformans. 
  • Role of mitofusin 2 mutations in the physiopathology of Charcot-Marie-Tooth disease type 2A. 
  • Role of sugars and organic acids in regulating the concentration and activity of the alternative oxidase in Poa annua roots. 
  • SIRT3 deficiency and mitochondrial protein hyperacetylation accelerate the development of the metabolic syndrome. 
  • SIRT3 regulates mitochondrial protein acetylation and intermediary metabolism. 
  • SIRT3 weighs heavily in the metabolic balance: a new role for SIRT3 in metabolic syndrome. 
  • SIRT3-dependent deacetylation exacerbates acetaminophen hepatotoxicity. 
  • SIRT4 Is a Lysine Deacylase that Controls Leucine Metabolism and Insulin Secretion. 
  • SIRT4 coordinates the balance between lipid synthesis and catabolism by repressing malonyl CoA decarboxylase. 
  • SIRT5 regulates the mitochondrial lysine succinylome and metabolic networks. 
  • Selective activation of mitogen-activated protein (MAP) kinase kinase 3 and p38alpha MAP kinase is essential for cyclic AMP-dependent UCP1 expression in adipocytes. 
  • Smac mimetic Birinapant induces apoptosis and enhances TRAIL potency in inflammatory breast cancer cells in an IAP-dependent and TNF-α-independent mechanism. 
  • Staphylococcus aureus sepsis induces early renal mitochondrial DNA repair and mitochondrial biogenesis in mice. 
  • Strain-specific response to beta 3-adrenergic receptor agonist treatment of diet-induced obesity in mice. 
  • Structural and functional consequences of coenzyme binding to the inactive asian variant of mitochondrial aldehyde dehydrogenase: roles of residues 475 and 487. 
  • Structure and function of the native and recombinant mitochondrial MRP1/MRP2 complex from Trypanosoma brucei. 
  • Structure-function relationships of the alternative oxidase of plant mitochondria: a model of the active site. 
  • Substrate availability and transcriptional regulation of metabolic genes in human skeletal muscle during recovery from exercise. 
  • Support for a monophyletic lemuriformes: overcoming incongruence between data partitions. 
  • Survival in critical illness is associated with early activation of mitochondrial biogenesis. 
  • TRPV4 is a regulator of adipose oxidative metabolism, inflammation, and energy homeostasis. 
  • Temporal dynamics of liver mitochondrial protein acetylation and succinylation and metabolites due to high fat diet and/or excess glucose or fructose. 
  • The Acetyl Group Buffering Action of Carnitine Acetyltransferase Offsets Macronutrient-Induced Lysine Acetylation of Mitochondrial Proteins. 
  • The Innate Immune Receptor NLRX1 Functions as a Tumor Suppressor by Reducing Colon Tumorigenesis and Key Tumor-Promoting Signals. 
  • The Mammalian-Specific Protein Armcx1 Regulates Mitochondrial Transport during Axon Regeneration. 
  • The active site of the cyanide-resistant oxidase from plant mitochondria contains a binuclear iron center. 
  • The alternative oxidase in roots of poa annua after transfer from high-light to low-light conditions. 
  • The alternative oxidase of plant mitochondria is involved in the acclimation of shoot growth at low temperature. A study of Arabidopsis AOX1a transgenic plants. 
  • The biological foundation of the genetic association of TOMM40 with late-onset Alzheimer's disease. 
  • The genetic architecture of biofilm formation in a clinical isolate of Saccharomyces cerevisiae. 
  • The mitochondrial cyanide-resistant oxidase: structural conservation amid regulatory diversity. 
  • The product of the alternative oxidase is still H2O. 
  • The relationship between the mitochondrial gene T-urf13 and fungal pathotoxin sensitivity in maize. 
  • Towards a new classification of the Arthoniales (Ascomycota) based on a three-gene phylogeny focussing on the genus Opegrapha. 
  • Transcriptional regulation of the mouse uncoupling protein-2 gene. Double E-box motif is required for peroxisome proliferator-activated receptor-gamma-dependent activation. 
  • Tumor necrosis factor increases mitochondrial oxidant production and induces expression of uncoupling protein-2 in the regenerating mice [correction of rat] liver. 
  • URF13, a ligand-gated, pore-forming receptor for T-toxin in the inner membrane of cms-T mitochondria. 
  • URF13, a maize mitochondrial pore-forming protein, is oligomeric and has a mixed orientation in Escherichia coli plasma membranes. 
  • Uncoupling protein-2: a novel gene linked to obesity and hyperinsulinemia. 
  • Variability of the SIRT3 gene, human silent information regulator Sir2 homologue, and survivorship in the elderly. 
  • beta-Adrenergic activation of p38 MAP kinase in adipocytes: cAMP induction of the uncoupling protein 1 (UCP1) gene requires p38 MAP kinase. 
  • p38 mitogen-activated protein kinase is the central regulator of cyclic AMP-dependent transcription of the brown fat uncoupling protein 1 gene. 
  • p53 overexpression in advanced-stage endometrial adenocarcinoma. 
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  Keywords of People

  • Berchuck, Andrew, James M. Ingram Professor of Gynecologic Oncology, Obstetrics and Gynecology, Gynecologic Oncology
  • Ferreira, Paulo Alexandre, Associate Professor in Ophthalmology, Pathology
  • Hirschey, Matthew, Associate Professor, Pharmacology & Cancer Biology