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 novel ubiquitin fusion system bypasses the mitochondria and generates biologically active Smac/DIABLO. 
  • 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. 
  • 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 candidate antagonists of IAP-mediated caspase inhibition using yeast reconstituted with the mammalian Apaf-1-activated apoptosis mechanism. 
  • Analysis of microsatellite mutations in the mitochondrial DNA of Saccharomyces cerevisiae. 
  • BCAA catabolism in brown fat controls energy homeostasis through SLC25A44. 
  • 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. 
  • CODAS syndrome is associated with mutations of LONP1, encoding mitochondrial AAA+ Lon protease. 
  • Characterization of an iron-sulfur cluster assembly protein (ISU1) from Schizosaccharomyces pombe. 
  • 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. 
  • 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. 
  • Dietary Sugars Alter Hepatic Fatty Acid Oxidation via Transcriptional and Post-translational Modifications of Mitochondrial Proteins. 
  • 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 Acetyl-Lysine Turnover in Muscle Mitochondria Promotes Insulin Resistance and Redox Stress without Overt Respiratory Dysfunction. 
  • 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 air pollution on mitochondrial function, mitochondrial DNA methylation, and mitochondrial peptide expression. 
  • Endothelial uncoupling protein 2 regulates mitophagy and pulmonary hypertension during intermittent hypoxia. 
  • 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. 
  • Enhanced cytoprotective effects of the inhibitor of apoptosis protein cellular IAP1 through stabilization with TRAF2. 
  • 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 mitochondrial membrane-linked SAB determines severity of sex-dependent acute liver injury. 
  • Expression of mitofusin 2(R94Q) in a transgenic mouse leads to Charcot-Marie-Tooth neuropathy type 2A. 
  • Extracellular Mitochondrial DNA and N-Formyl Peptides in Trauma and Critical Illness: A Systematic Review. 
  • Extreme Acetylation of the Cardiac Mitochondrial Proteome Does Not Promote Heart Failure. 
  • 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. 
  • Foxa1-deficient mice exhibit impaired insulin secretion due to uncoupled oxidative phosphorylation. 
  • Genome-Wide Association Meta-Analysis Reveals Novel Juvenile Idiopathic Arthritis Susceptibility Loci. 
  • Genomic characterization of POS5, the Saccharomyces cerevisiae mitochondrial NADH kinase. 
  • 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. 
  • Hepatic Mitochondrial SAB Deletion or Knockdown Alleviates Diet-Induced Metabolic Syndrome, Steatohepatitis, and Hepatic Fibrosis. 
  • Human pluripotent stem cells decouple respiration from energy production. 
  • IAP proteins: sticking it to Smac. 
  • 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. 
  • Induction by leptin of uncoupling protein-2 and enzymes of fatty acid oxidation. 
  • Inherited variants in mitochondrial biogenesis genes may influence epithelial ovarian cancer risk. 
  • Inhibitor of apoptosis proteins as intracellular signaling intermediates. 
  • 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 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. 
  • Mammalian Sir2 homolog SIRT3 regulates global mitochondrial lysine acetylation. 
  • Manganese porphyrin reduces renal injury and mitochondrial damage during ischemia/reperfusion. 
  • Metabolic flexibility via mitochondrial BCAA carrier SLC25A44 is required for optimal fever. 
  • Method for the structural analysis of Twinkle mitochondrial DNA helicase by cryo-EM. 
  • Microparticles in the blood of patients with SLE: Size, content of mitochondria and role in circulating immune complexes. 
  • Mitochondrial CaMKII causes adverse metabolic reprogramming and dilated cardiomyopathy. 
  • 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 microproteins link metabolic cues to respiratory chain biogenesis. 
  • Mitochondrial protein acetylation regulates metabolism. 
  • Mitochondrial protein adduct and superoxide generation are prerequisites for early activation of c-jun N-terminal kinase within the cytosol after an acetaminophen overdose in mice. 
  • Mitochondrial protein adducts formation and mitochondrial dysfunction during N-acetyl-m-aminophenol (AMAP)-induced hepatotoxicity in primary human hepatocytes. 
  • 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. 
  • Muscle oxidative capacity during IL-6-dependent cancer cachexia. 
  • Mutations in SCO2 are associated with autosomal-dominant high-grade myopia. 
  • Mutations in TFAM, encoding mitochondrial transcription factor A, cause neonatal liver failure associated with mtDNA depletion. 
  • 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. 
  • 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. 
  • 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. 
  • 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 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. 
  • RBFOX2 is critical for maintaining alternative polyadenylation patterns and mitochondrial health in rat myoblasts. 
  • 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. 
  • Regulation of UCP1 and Mitochondrial Metabolism in Brown Adipose Tissue by Reversible Succinylation. 
  • Regulation of mitochondrial morphology by APC/CCdh1-mediated control of Drp1 stability. 
  • Regulation of mitochondrial processes by protein S-nitrosylation. 
  • Regulation of skeletal muscle oxidative capacity and insulin signaling by the mitochondrial rhomboid protease PARL. 
  • 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. 
  • Remodeling of the Acetylproteome by SIRT3 Manipulation Fails to Affect Insulin Secretion or β Cell Metabolism in the Absence of Overnutrition. 
  • 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. 
  • 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. 
  • 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. 
  • SSBP1 mutations cause mtDNA depletion underlying a complex optic atrophy disorder. 
  • 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. 
  • Smac/Diablo antagonizes ubiquitin ligase activity of inhibitor of apoptosis proteins. 
  • 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 insight and characterization of human Twinkle helicase in mitochondrial disease. 
  • Structure and function of the native and recombinant mitochondrial MRP1/MRP2 complex from Trypanosoma brucei. 
  • Structures of AAA protein translocase Bcs1 suggest translocation mechanism of a folded protein. 
  • 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 Arabidopsis sn-1-specific mitochondrial acylhydrolase AtDLAH is positively correlated with seed viability. 
  • The Mammalian-Specific Protein Armcx1 Regulates Mitochondrial Transport during Axon Regeneration. 
  • The binding of SLE autoantibodies to mitochondria. 
  • 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. 
  • Thyroid hormone receptor and ERRα coordinately regulate mitochondrial fission, mitophagy, biogenesis, and function. 
  • Titin truncations lead to impaired cardiomyocyte autophagy and mitochondrial function in vivo. 
  • 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. 
  • UCP2-induced fatty acid synthase promotes NLRP3 inflammasome activation during sepsis. 
  • Uncoupling protein-2: a novel gene linked to obesity and hyperinsulinemia. 
  • Upstream regulatory role for XIAP in receptor-mediated apoptosis. 
  • Variability of the SIRT3 gene, human silent information regulator Sir2 homologue, and survivorship in the elderly. 
  • XIAP inhibition of caspase-3 preserves its association with the Apaf-1 apoptosome and prevents CD95- and Bax-induced apoptosis. 
  • 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. 
  • β-Cell-specific ablation of sirtuin 4 does not affect nutrient-stimulated insulin secretion in mice. 
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  Keywords of People

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