Search Results - "Burr, Stephen"

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  1. 1

    The mitochondrial DNA genetic bottleneck: inheritance and beyond by Zhang, Haixin, Burr, Stephen P, Chinnery, Patrick F

    Published in Essays in biochemistry (20-07-2018)
    “…mtDNA is a multicopy genome. When mutations exist, they can affect a varying proportion of the mtDNA present within every cell (heteroplasmy). Heteroplasmic…”
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  2. 2

    Origins of tissue and cell-type specificity in mitochondrial DNA (mtDNA) disease by Burr, Stephen P, Chinnery, Patrick F

    Published in Human molecular genetics (22-05-2024)
    “…Abstract Mutations of mitochondrial (mt)DNA are a major cause of morbidity and mortality in humans, accounting for approximately two thirds of diagnosed…”
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  3. 3

    Mitochondria and Hypoxia: Metabolic Crosstalk in Cell-Fate Decisions by Bargiela, David, Burr, Stephen P., Chinnery, Patrick F.

    Published in Trends in endocrinology and metabolism (01-04-2018)
    “…Alterations in mitochondrial metabolism influence cell differentiation and growth. This process is regulated by the activity of 2-oxoglutarate (2OG)-dependent…”
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  4. 4

    Mitochondrial Protein Lipoylation and the 2-Oxoglutarate Dehydrogenase Complex Controls HIF1α Stability in Aerobic Conditions by Burr, Stephen P., Costa, Ana S.H., Grice, Guinevere L., Timms, Richard T., Lobb, Ian T., Freisinger, Peter, Dodd, Roger B., Dougan, Gordon, Lehner, Paul J., Frezza, Christian, Nathan, James A.

    Published in Cell metabolism (08-11-2016)
    “…Hypoxia-inducible transcription factors (HIFs) control adaptation to low oxygen environments by activating genes involved in metabolism, angiogenesis, and…”
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    Drought-Mediated Changes in Tree Physiological Processes Weaken Tree Defenses to Bark Beetle Attack by Kolb, Thomas, Keefover-Ring, Ken, Burr, Stephen J., Hofstetter, Richard, Gaylord, Monica, Raffa, Kenneth F.

    Published in Journal of chemical ecology (01-10-2019)
    “…Interactions between water stress and induced defenses and their role in tree mortality due to bark beetles are poorly understood. We performed a factorial…”
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    Mitochondrial DNA Heteroplasmy and Purifying Selection in the Mammalian Female Germ Line by Burr, Stephen P., Pezet, Mikael, Chinnery, Patrick F.

    Published in Development, growth & differentiation (01-01-2018)
    “…Inherited mutations in the mitochondrial (mt)DNA are a major cause of human disease, with approximately 1 in 5000 people affected by one of the hundreds of…”
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  7. 7

    Mesenchymal stromal cells and regulatory T cells: the Yin and Yang of peripheral tolerance? by Burr, Stephen P, Dazzi, Francesco, Garden, Oliver A

    Published in Immunology and cell biology (01-01-2013)
    “…In recent years, mesenchymal stromal cells (MSCs) and regulatory T cells (Tregs) have both garnered significant interest from immunologists worldwide, not…”
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  8. 8

    LRPPRC and SLIRP synergize to maintain sufficient and orderly mammalian mitochondrial translation by Rubalcava-Gracia, Diana, Bubb, Kristina, Levander, Fredrik, Burr, Stephen P, August, Amelie V, Chinnery, Patrick F, Koolmeister, Camilla, Larsson, Nils-Göran

    Published in Nucleic acids research (14-10-2024)
    “…Abstract In mammals, the leucine-rich pentatricopeptide repeat protein (LRPPRC) and the stem-loop interacting RNA-binding protein (SLIRP) form a complex in the…”
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  9. 9

    The vacuolar-ATPase complex and assembly factors, TMEM199 and CCDC115, control HIF1α prolyl hydroxylation by regulating cellular iron levels by Miles, Anna L, Burr, Stephen P, Grice, Guinevere L, Nathan, James A

    Published in eLife (15-03-2017)
    “…Hypoxia Inducible transcription Factors (HIFs) are principally regulated by the 2-oxoglutarate and Iron(II) prolyl hydroxylase (PHD) enzymes, which hydroxylate…”
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  10. 10

    The leaf miner Phyllocnistis populiella negatively impacts water relations in aspen by Wagner, Diane, Wheeler, Jenifer M, Burr, Stephen J

    Published in Tree physiology (11-05-2020)
    “…Within the North American boreal forest, a widespread outbreak of the epidermal leaf miner Phyllocnistis populiella Cham. has damaged quaking aspen (Populus…”
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  11. 11

    MARCH6 and TRC8 facilitate the quality control of cytosolic and tail‐anchored proteins by Stefanovic‐Barrett, Sandra, Dickson, Anna S, Burr, Stephen P, Williamson, James C, Lobb, Ian T, van den Boomen, Dick JH, Lehner, Paul J, Nathan, James A

    Published in EMBO reports (01-05-2018)
    “…Misfolded or damaged proteins are typically targeted for destruction by proteasome‐mediated degradation, but the mammalian ubiquitin machinery involved is…”
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  12. 12

    ABHD11 maintains 2-oxoglutarate metabolism by preserving functional lipoylation of the 2-oxoglutarate dehydrogenase complex by Bailey, Peter S. J., Ortmann, Brian M., Martinelli, Anthony W., Houghton, Jack W., Costa, Ana S. H., Burr, Stephen P., Antrobus, Robin, Frezza, Christian, Nathan, James A.

    Published in Nature communications (13-08-2020)
    “…2-oxoglutarate (2-OG or α-ketoglutarate) relates mitochondrial metabolism to cell function by modulating the activity of 2-OG dependent dioxygenases involved…”
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  13. 13

    MTFP1 controls mitochondrial fusion to regulate inner membrane quality control and maintain mtDNA levels by Tábara, Luis Carlos, Burr, Stephen P, Frison, Michele, Chowdhury, Suvagata R, Paupe, Vincent, Nie, Yu, Johnson, Mark, Villar-Azpillaga, Jara, Viegas, Filipa, Segawa, Mayuko, Anand, Hanish, Petkevicius, Kasparas, Chinnery, Patrick F, Prudent, Julien

    Published in Cell (11-07-2024)
    “…Mitochondrial dynamics play a critical role in cell fate decisions and in controlling mtDNA levels and distribution. However, the molecular mechanisms linking…”
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    Density of Emerald Ash Borer (Coleoptera: Buprestidae) Adults and Larvae at Three Stages of the Invasion Wave by Burr, Stephen J, McCullough, Deborah G, Poland, Therese M

    Published in Environmental entomology (08-02-2018)
    “…Emerald ash borer (EAB) (Agrilus planipennis Fairmaire) (Coleoptera: Buprestidae), an invasive phloem-feeding buprestid, has killed hundreds of millions of ash…”
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    Mitochondrial DNA heteroplasmy is modulated during oocyte development propagating mutation transmission by Zhang, Haixin, Esposito, Marco, Pezet, Mikael G, Aryaman, Juvid, Wei, Wei, Klimm, Florian, Calabrese, Claudia, Burr, Stephen P, Macabelli, Carolina H, Viscomi, Carlo, Saitou, Mitinori, Chiaratti, Marcos R, Stewart, James B, Jones, Nick, Chinnery, Patrick F

    Published in Science advances (10-12-2021)
    “…Heteroplasmic mitochondrial DNA (mtDNA) mutations are a common cause of inherited disease, but a few recurrent mutations account for the vast majority of new…”
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    Oxygen tension modulates the mitochondrial genetic bottleneck and influences the segregation of a heteroplasmic mtDNA variant in vitro by Pezet, Mikael G., Gomez-Duran, Aurora, Klimm, Florian, Aryaman, Juvid, Burr, Stephen, Wei, Wei, Saitou, Mitinori, Prudent, Julien, Chinnery, Patrick F.

    Published in Communications biology (14-05-2021)
    “…Most humans carry a mixed population of mitochondrial DNA (mtDNA heteroplasmy) affecting ~1–2% of molecules, but rapid percentage shifts occur over one…”
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