| scholarly article | Q13442814 |
| P2093 | author name string | Peter J McGuire | |
| Senta M Kapnick | |||
| Susan E Pacheco | |||
| P2860 | cites work | OXPHOS gene expression and control in mitochondrial disorders. | Q37457220 |
| Targeted expression of the human uncoupling protein 2 (hUCP2) to adult neurons extends life span in the fly. | Q46626387 | ||
| A spontaneous mutation in the nicotinamide nucleotide transhydrogenase gene of C57BL/6J mice results in mitochondrial redox abnormalities. | Q46719392 | ||
| Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism | Q46733619 | ||
| Dynamin related protein 1-dependent mitochondrial fission regulates oxidative signalling in T cells | Q46912618 | ||
| Association between uncoupling protein polymorphisms (UCP2-UCP3) and energy metabolism/obesity in Pima indians. | Q47700926 | ||
| Association of UCP2 -866 G/A polymorphism with chronic inflammatory diseases | Q47959866 | ||
| Mitochondrial transcription factor A regulates mtDNA copy number in mammals | Q47963219 | ||
| Integrative Proteomics and Phosphoproteomics Profiling Reveals Dynamic Signaling Networks and Bioenergetics Pathways Underlying T Cell Activation. | Q50451267 | ||
| The dynamics of mitochondrial DNA heteroplasmy: implications for human health and disease. | Q50994607 | ||
| Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression. | Q52533533 | ||
| Mitochondria take up Ca2+ in two steps dependently on store-operated Ca2+ entry in mast cells. | Q52741410 | ||
| Mitochondrial uncoupling protein 2 is up-regulated in human head and neck, skin, pancreatic, and prostate tumors. | Q54338934 | ||
| INVASIVE ASPERGILLOSIS IN TWO PATIENTS WITH PEARSON SYNDROME | Q58839994 | ||
| Somatic Progenitor Cell Vulnerability to Mitochondrial DNA Mutagenesis Underlies Progeroid Phenotypes in Polg Mutator Mice | Q59545994 | ||
| A mouse model for mitochondrial myopathy and cardiomyopathy resulting from a deficiency in the heart/muscle isoform of the adenine nucleotide translocator | Q61874674 | ||
| Predisposition to infection and SIRS in mitochondrial disorders: 8 years' experience in an academic center | Q63979798 | ||
| Elevated glucose concentrations inhibit DNA synthesis and expression of c-myc in cultured hepatocytes | Q72128921 | ||
| The shift in the myocardial adenine nucleotide translocator isoform expression pattern is associated with an enteroviral infection in the absence of an active T-cell dependent immune response in human inflammatory heart disease | Q73858805 | ||
| Ca ion uptake by rat kidney mitochondria and its dependence on respiration and phosphorylation | Q79434435 | ||
| Increased longevity and refractoriness to Ca(2+)-dependent neurodegeneration in Surf1 knockout mice | Q79518243 | ||
| Opa1 deficiency in a mouse model of autosomal dominant optic atrophy impairs mitochondrial morphology, optic nerve structure and visual function | Q80146686 | ||
| The uncoupling protein 2 modulates the cytokine balance in innate immunity | Q80257595 | ||
| Mitochondria positioning controls local calcium influx in T cells | Q84965190 | ||
| SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome | Q22008476 | ||
| A mutation in the human heme A:farnesyltransferase gene (COX10 ) causes cytochrome c oxidase deficiency | Q22253915 | ||
| Mitochondrial DNA stress primes the antiviral innate immune response | Q23030015 | ||
| Premature ageing in mice expressing defective mitochondrial DNA polymerase | Q24294365 | ||
| MICU1 encodes a mitochondrial EF hand protein required for Ca(2+) uptake | Q24294670 | ||
| The mitochondrial transcription and packaging factor Tfam imposes a U-turn on mitochondrial DNA | Q24294939 | ||
| LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs | Q24295176 | ||
| Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patients | Q24297605 | ||
| Integrative genomics identifies MCU as an essential component of the mitochondrial calcium uniporter | Q24306825 | ||
| Mutations in COX10 result in a defect in mitochondrial heme A biosynthesis and account for multiple, early-onset clinical phenotypes associated with isolated COX deficiency | Q24317097 | ||
| Mutations in FBXL4, encoding a mitochondrial protein, cause early-onset mitochondrial encephalomyopathy | Q24321305 | ||
| Uncoupling protein-2: a novel gene linked to obesity and hyperinsulinemia | Q24324798 | ||
| MICU1 and MICU2 finely tune the mitochondrial Ca2+ uniporter by exerting opposite effects on MCU activity | Q24338031 | ||
| Cardiac and skeletal muscle defects in a mouse model of human Barth syndrome | Q24598420 | ||
| Understanding the Warburg effect: the metabolic requirements of cell proliferation | Q24604760 | ||
| Age-associated mosaic respiratory chain deficiency causes trans-neuronal degeneration | Q24646438 | ||
| Endosymbiotic theories for eukaryote origin | Q26795689 | ||
| MTO1-deficient mouse model mirrors the human phenotype showing complex I defect and cardiomyopathy | Q27320993 | ||
| DNA wrapping and bending by a mitochondrial high mobility group-like transcriptional activator protein | Q27933227 | ||
| Disruption of the uncoupling protein-2 gene in mice reveals a role in immunity and reactive oxygen species production | Q28139177 | ||
| Mutant mitochondrial thymidine kinase in mitochondrial DNA depletion myopathy | Q28203066 | ||
| Clinical and genetic spectrum of mitochondrial neurogastrointestinal encephalomyopathy | Q28248577 | ||
| Dilated cardiomyopathy and neonatal lethality in mutant mice lacking manganese superoxide dismutase | Q28284593 | ||
| A mutation in Orai1 causes immune deficiency by abrogating CRAC channel function | Q28305026 | ||
| Mitochondrial complex I deficiency leads to inflammation and retinal ganglion cell death in the Ndufs4 mouse | Q28392327 | ||
| The mitochondrial fusion-related proteins Mfn2 and OPA1 are transcriptionally induced during differentiation of bone marrow progenitors to immature dendritic cells | Q35038993 | ||
| The dynamin-related GTPase Opa1 is required for glucose-stimulated ATP production in pancreatic beta cells | Q35083432 | ||
| Underexpression of mitochondrial-DNA encoded ATP synthesis-related genes and DNA repair genes in systemic lupus erythematosus | Q35093017 | ||
| Genetic modification of survival in tissue-specific knockout mice with mitochondrial cardiomyopathy | Q35112849 | ||
| Attenuation of experimental colitis in glutathione peroxidase 1 and catalase double knockout mice through enhancing regulatory T cell function | Q35151625 | ||
| Mitochondrial Diseases Part I: mouse models of OXPHOS deficiencies caused by defects in respiratory complex subunits or assembly factors | Q35190973 | ||
| Progressive parkinsonism in mice with respiratory-chain-deficient dopamine neurons | Q35611727 | ||
| Mitochondrial Diseases Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function | Q35660977 | ||
| UCP2 knockout suppresses mouse skin carcinogenesis. | Q35674783 | ||
| VDAC1 selectively transfers apoptotic Ca2+ signals to mitochondria. | Q35687111 | ||
| Decreased mtDNA, oxidative stress, cardiomyopathy, and death from transgenic cardiac targeted human mutant polymerase gamma. | Q35695170 | ||
| The Mitochondrial Calcium Uniporter Selectively Matches Metabolic Output to Acute Contractile Stress in the Heart | Q35836414 | ||
| Mitochondrial ATP transporter Ant2 depletion impairs erythropoiesis and B lymphopoiesis | Q35941463 | ||
| T cell activation requires mitochondrial translocation to the immunological synapse | Q35971606 | ||
| Diet-induced changes in uncoupling proteins in obesity-prone and obesity-resistant strains of mice | Q36017740 | ||
| Regulation of NKT cell-mediated immune responses to tumours and liver inflammation by mitochondrial PGAM5-Drp1 signalling. | Q36077924 | ||
| Leukocyte mitochondrial DNA alteration in systemic lupus erythematosus and its relevance to the susceptibility to lupus nephritis | Q36197323 | ||
| Mechanism of Activation-Induced Downregulation of Mitofusin 2 in Human Peripheral Blood T Cells | Q36349560 | ||
| Calcium uptake by rat kidney mitochondria | Q36441249 | ||
| Ca2+ signals regulate mitochondrial metabolism by stimulating CREB-mediated expression of the mitochondrial Ca2+ uniporter gene MCU. | Q36490057 | ||
| Mitochondria are required for antigen-specific T cell activation through reactive oxygen species signaling. | Q36639793 | ||
| MICU1 regulation of mitochondrial Ca(2+) uptake dictates survival and tissue regeneration. | Q36675219 | ||
| Aberrant Schwann cell lipid metabolism linked to mitochondrial deficits leads to axon degeneration and neuropathy. | Q36676245 | ||
| Metabolic regulation of T lymphocytes | Q36711431 | ||
| Mitochondrial content is central to nuclear gene expression: Profound implications for human health. | Q36762165 | ||
| Metabolic switching and fuel choice during T-cell differentiation and memory development | Q36820974 | ||
| Glutathione peroxidase 1 deficiency attenuates concanavalin A-induced hepatic injury by modulation of T-cell activation | Q36866499 | ||
| Uncoupling protein 2 regulates metabolic reprogramming and fate of antigen-stimulated CD8+ T cells. | Q37033733 | ||
| CD8 memory T cells have a bioenergetic advantage that underlies their rapid recall ability. | Q37143600 | ||
| Inhibiting glycolytic metabolism enhances CD8+ T cell memory and antitumor function. | Q37200886 | ||
| Mitochondrial cristae shape determines respiratory chain supercomplexes assembly and respiratory efficiency | Q37215027 | ||
| Mitochondrial Respiration Controls Lysosomal Function during Inflammatory T Cell Responses. | Q37262107 | ||
| Redox and reactive oxygen species regulation of mitochondrial cytochrome C oxidase biogenesis | Q37366433 | ||
| Mitofusin 2 maintains haematopoietic stem cells with extensive lymphoid potential | Q37410531 | ||
| Thymidine kinase 2 (H126N) knockin mice show the essential role of balanced deoxynucleotide pools for mitochondrial DNA maintenance. | Q28504546 | ||
| Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency | Q28512560 | ||
| LRPPRC mutation suppresses cytochrome oxidase activity by altering mitochondrial RNA transcript stability in a mouse model | Q28512618 | ||
| Progressive loss of mitochondrial DNA in thymidine kinase 2-deficient mice | Q28513054 | ||
| Deficiency in Cardiolipin Reduces Doxorubicin-Induced Oxidative Stress and Mitochondrial Damage in Human B-Lymphocytes | Q28552702 | ||
| Ovarian function in superoxide dismutase 1 and 2 knockout mice | Q28593231 | ||
| Mitochondrial oxidative stress in mice lacking the glutathione peroxidase-1 gene | Q28593572 | ||
| Mitofusins Mfn1 and Mfn2 coordinately regulate mitochondrial fusion and are essential for embryonic development | Q28594513 | ||
| Autophagy proteins regulate innate immune responses by inhibiting the release of mitochondrial DNA mediated by the NALP3 inflammasome | Q29547419 | ||
| Circulating mitochondrial DAMPs cause inflammatory responses to injury | Q29614470 | ||
| Mitochondrial DNA mutations, oxidative stress, and apoptosis in mammalian aging | Q29616056 | ||
| Mitochondrial fission, fusion, and stress | Q29616536 | ||
| Mitochondria and cancer | Q29616610 | ||
| Mitochondrial fission factor Drp1 is essential for embryonic development and synapse formation in mice | Q29617092 | ||
| The machines that divide and fuse mitochondria | Q29617094 | ||
| Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice | Q29619811 | ||
| Oxidized mitochondrial DNA activates the NLRP3 inflammasome during apoptosis | Q29619852 | ||
| ER tubules mark sites of mitochondrial division | Q29619991 | ||
| Physiological roles of mitochondrial reactive oxygen species | Q29620297 | ||
| Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathy | Q30484862 | ||
| The mitochondrial fission factor dynamin-related protein 1 modulates T-cell receptor signalling at the immune synapse | Q30500233 | ||
| Fatal neonatal-onset mitochondrial respiratory chain disease with T cell immunodeficiency. | Q33372029 | ||
| Hematological manifestations of primary mitochondrial disorders | Q33375931 | ||
| STIM1 mutation associated with a syndrome of immunodeficiency and autoimmunity | Q33384395 | ||
| Mature T lymphocyte apoptosis--immune regulation in a dynamic and unpredictable antigenic environment | Q33652471 | ||
| Accumulating mitochondrial DNA mutations drive premature hematopoietic aging phenotypes distinct from physiological stem cell aging | Q33891793 | ||
| Distribution and medical impact of loss-of-function variants in the Finnish founder population | Q33981677 | ||
| Role of uncoupling protein UCP2 in cell-mediated immunity: how macrophage-mediated insulitis is accelerated in a model of autoimmune diabetes. | Q34008199 | ||
| Mitochondrial complex I activity suppresses inflammation and enhances bone resorption by shifting macrophage-osteoclast polarization | Q34143332 | ||
| Mutant mitochondrial helicase Twinkle causes multiple mtDNA deletions and a late-onset mitochondrial disease in mice | Q34201844 | ||
| Mitochondrial adenine nucleotide translocator 3 is regulated by IL-4 and IFN-gamma via STAT-dependent pathways | Q34292946 | ||
| Cytochrome c oxidase biogenesis in a patient with a mutation in COX10 gene. | Q34354144 | ||
| Mouse models for mitochondrial disease | Q34386758 | ||
| Elevated mitochondrial superoxide disrupts normal T cell development, impairing adaptive immune responses to an influenza challenge | Q34516742 | ||
| Viable RNaseH1 knockout mice show RNaseH1 is essential for R loop processing, mitochondrial and liver function | Q34524329 | ||
| Neutrophils in Barth syndrome (BTHS) avidly bind annexin-V in the absence of apoptosis | Q34545022 | ||
| The otolaryngological manifestations of mitochondrial disease and the risk of neurodegeneration with infection | Q34583672 | ||
| Animal models for mitochondrial disease | Q34639988 | ||
| UCP2, not a physiologically relevant uncoupler but a glucose sparing switch impacting ROS production and glucose sensing | Q37469361 | ||
| UCP2 overexpression worsens mitochondrial dysfunction and accelerates disease progression in a mouse model of amyotrophic lateral sclerosis | Q37478060 | ||
| UCP2 transports C4 metabolites out of mitochondria, regulating glucose and glutamine oxidation. | Q37519690 | ||
| miRNA-133a-UCP2 pathway regulates inflammatory bowel disease progress by influencing inflammation, oxidative stress and energy metabolism | Q37572580 | ||
| MICU1 Serves as a Molecular Gatekeeper to Prevent In Vivo Mitochondrial Calcium Overload | Q37651609 | ||
| Intracellular Ca2+ storage in health and disease: a dynamic equilibrium. | Q37699891 | ||
| Mast cells and immunoregulation/immunomodulation. | Q37895548 | ||
| Mitochondrial uncoupling protein 2 (UCP2) in glucose and lipid metabolism | Q37932159 | ||
| New aspects of mitochondrial Uncoupling Proteins (UCPs) and their roles in tumorigenesis | Q37939833 | ||
| Mitochondrial regulation of cell cycle and proliferation | Q37941864 | ||
| Mitochondrial disorders as windows into an ancient organelle | Q38059778 | ||
| Metabolic pathways in immune cell activation and quiescence | Q38100361 | ||
| Cytochrome c Oxidase Activity Is a Metabolic Checkpoint that Regulates Cell Fate Decisions During T Cell Activation and Differentiation | Q38737991 | ||
| Impaired expression of the mitochondrial calcium uniporter suppresses mast cell degranulation | Q38836812 | ||
| Mouse models for mitochondrial diseases. | Q38872626 | ||
| Tissue-specific loss of DARS2 activates stress responses independently of respiratory chain deficiency in the heart | Q39017553 | ||
| Barth syndrome: cellular compensation of mitochondrial dysfunction and apoptosis inhibition due to changes in cardiolipin remodeling linked to tafazzin (TAZ) gene mutation | Q39176896 | ||
| Cause of Death in Children With Mitochondrial Diseases | Q39188617 | ||
| The effect of calcium on the respiratory and phosphorylative activities of heart-muscle sarcosomes | Q39227221 | ||
| Adipose-specific deletion of TFAM increases mitochondrial oxidation and protects mice against obesity and insulin resistance | Q39241662 | ||
| The effects of calcium ions and adenine nucleotides on the activity of pig heart 2-oxoglutarate dehydrogenase complex | Q39273223 | ||
| Nicotinamide nucleotide transhydrogenase (NNT) acts as a novel modulator of macrophage inflammatory responses | Q39346888 | ||
| The cellular and molecular mechanisms for neutropenia in Barth syndrome | Q39453643 | ||
| The human as an experimental system in molecular genetics | Q39643714 | ||
| Mitochondrial Dynamics Controls T Cell Fate through Metabolic Programming. | Q39689532 | ||
| Calcium ions and the regulation of NAD+-linked isocitrate dehydrogenase from the mitochondria of rat heart and other tissues | Q40215031 | ||
| Characterization of lymphoblast mitochondria from patients with Barth syndrome | Q40439645 | ||
| Sequential waves of functionally related proteins are expressed when B cells prepare for antibody secretion | Q40669445 | ||
| Mitochondrial respiratory capacity is a critical regulator of CD8+ T cell memory development | Q40760682 | ||
| Respiratory chain encephalomyopathies: a diagnostic classification | Q41158319 | ||
| Evidence of Oxidative Stress and Secondary Mitochondrial Dysfunction in Metabolic and Non-Metabolic Disorders | Q41161393 | ||
| MICU1 controls both the threshold and cooperative activation of the mitochondrial Ca²⁺ uniporter | Q41976396 | ||
| Association of a genetic variant of carnitine palmitoyltransferase 1A with infections in Alaska Native children | Q42249231 | ||
| Role of calcium ions in the regulation of intramitochondrial metabolism. Effects of Na+, Mg2+ and ruthenium red on the Ca2+-stimulated oxidation of oxoglutarate and on pyruvate dehydrogenase activity in intact rat heart mitochondria | Q42881253 | ||
| Effect of micromolar concentrations of free Ca2+ ions on pyruvate dehydrogenase interconversion in intact rat heart mitochondria | Q42883694 | ||
| Expression of mitofusin 2(R94Q) in a transgenic mouse leads to Charcot-Marie-Tooth neuropathy type 2A. | Q43086039 | ||
| Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice. | Q43757469 | ||
| Loss-of-function mutations in MICU1 cause a brain and muscle disorder linked to primary alterations in mitochondrial calcium signaling | Q44468436 | ||
| Glutathione peroxidase-1 reduces influenza A virus-induced lung inflammation. | Q44990480 | ||
| Uncoupling protein-2 is critical for nigral dopamine cell survival in a mouse model of Parkinson's disease. | Q45213390 | ||
| Mitochondrial fusion protects against neurodegeneration in the cerebellum. | Q45931731 | ||
| Increased mitochondrial Ca2+ and decreased sarcoplasmic reticulum Ca2+ in mitochondrial myopathy | Q46302159 | ||
| UCP2 up-regulation within the course of autoimmune encephalomyelitis correlates with T-lymphocyte activation. | Q46426667 | ||
| P577 | publication date | 2017-11-18 | |
| P1433 | published in | Metabolism | Q15764354 |
| P1476 | title | The emerging role of immune dysfunction in mitochondrial diseases as a paradigm for understanding immunometabolism |
| Q90702003 | A New Approach to Treating Neurodegenerative Otologic Disorders |
| Q98772842 | Clinical and Immunological Characterization of Combined Immunodeficiency Due to TFRC Mutation in Eight Patients |
| Q92311624 | Defects of mitochondrial RNA turnover lead to the accumulation of double-stranded RNA in vivo |
| Q91741481 | Drosophila as a Model System to Investigate the Effects of Mitochondrial Variation on Innate Immunity |
| Q54234131 | Fatty acid metabolism in CD8+ T cell memory: Challenging current concepts. |
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