Search Results - "ROBINSON, Brian H"

Mutations in Iron-Sulfur Cluster Scaffold Genes NFU1 and BOLA3 Cause a Fatal Deficiency of Multiple Respiratory Chain and 2-Oxoacid Dehydrogenase Enzymes by Cameron, Jessie M., Janer, Alexandre, Levandovskiy, Valeriy, MacKay, Nevena, Rouault, Tracey A., Tong, Wing-Hang, Ogilvie, Isla, Shoubridge, Eric A., Robinson, Brian H.

“…Severe combined deficiency of the 2-oxoacid dehydrogenases, associated with a defect in lipoate synthesis and accompanied by defects in complexes I, II, and…”
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Inhibition of Mitochondrial Translation as a Therapeutic Strategy for Human Acute Myeloid Leukemia by Škrtić, Marko, Sriskanthadevan, Shrivani, Jhas, Bozhena, Gebbia, Marinella, Wang, Xiaoming, Wang, Zezhou, Hurren, Rose, Jitkova, Yulia, Gronda, Marcela, Maclean, Neil, Lai, Courteney K., Eberhard, Yanina, Bartoszko, Justyna, Spagnuolo, Paul, Rutledge, Angela C., Datti, Alessandro, Ketela, Troy, Moffat, Jason, Robinson, Brian H., Cameron, Jessie H., Wrana, Jeffery, Eaves, Connie J., Minden, Mark D., Wang, Jean C.Y., Dick, John E., Humphries, Keith, Nislow, Corey, Giaever, Guri, Schimmer, Aaron D.

“…To identify FDA-approved agents targeting leukemic cells, we performed a chemical screen on two human leukemic cell lines and identified the antimicrobial…”
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Latitudinal gradients and sex differences in morphology of the Black Oystercatcher (Haematopus bachmani) by Roodenrijs, Hannah, Ware, Lena, Rankin, Cole, Maftei, Mark, Hipfner, J. Mark, Robinson, Brian H., Esler, Daniel, Coletti, Heather, Green, David J.

“…Environment and behavior are widely understood to affect bird morphology, which can lead to differences among subspecies or populations within a wide‐ranging…”
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Synthesis, physiochemical characterization, and biocompatibility of a chitosan/dextran-based hydrogel for postsurgical adhesion prevention by Cabral, Jaydee D., Roxburgh, Marina, Shi, Zheng, Liu, Liqi, McConnell, Michelle, Williams, Gail, Evans, Natasha, Hanton, Lyall R., Simpson, Jim, Moratti, Stephen C., Robinson, Brian H., Wormald, Peter J., Robinson, Simon

“…An amine-functionalized succinyl chitosan and an oxidized dextran were synthesized and mixed in aqueous solution to form an in situ chitosan/dextran…”
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The role of the LRPPRC (leucine-rich pentatricopeptide repeat cassette) gene in cytochrome oxidase assembly: mutation causes lowered levels of COX (cytochrome c oxidase) I and COX III mRNA by Xu, Fenghao, Morin, Charles, Mitchell, Grant, Ackerley, Cameron, Robinson, Brian H

“…Leigh syndrome French Canadian (LSFC) is a variant of cytochrome oxidase deficiency found in Québec and caused by mutations in the LRPPRC (leucine-rich…”
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LRPPRC mutation suppresses cytochrome oxidase activity by altering mitochondrial RNA transcript stability in a mouse model by Xu, Fenghao, Addis, Jane B L, Cameron, Jessie M, Robinson, Brian H

“…LRPPRC (leucine-rich pentatricopeptide repeat-containing) has been shown to be essential for the maturation of COX (cytochrome c oxidase), possibly by…”
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Disruption of a mitochondrial RNA-binding protein gene results in decreased cytochrome b expression and a marked reduction in ubiquinol-cytochrome c reductase activity in mouse heart mitochondria by Xu, Fenghao, Ackerley, Cameron, Maj, Mary C, Addis, Jane B L, Levandovskiy, Valeriy, Lee, Jisoo, Mackay, Nevena, Cameron, Jessie M, Robinson, Brian H

“…Mice homozygous for a defect in the PTCD2 (pentatricopeptide repeat domain protein 2) gene were generated in order to study the role of this protein in…”
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Genome-wide association analysis identifies a mutation in the thiamine transporter 2 (SLC19A3) gene associated with Alaskan Husky encephalopathy by Vernau, Karen M, Runstadler, Jonathan A, Brown, Emily A, Cameron, Jessie M, Huson, Heather J, Higgins, Robert J, Ackerley, Cameron, Sturges, Beverly K, Dickinson, Peter J, Puschner, Birgit, Giulivi, Cecilia, Shelton, G Diane, Robinson, Brian H, DiMauro, Salvatore, Bollen, Andrew W, Bannasch, Danika L

“…Alaskan Husky Encephalopathy (AHE) has been previously proposed as a mitochondrial encephalopathy based on neuropathological similarities with human Leigh…”
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Diagnostic Accuracy of Blood Lactate-to-Pyruvate Molar Ratio in the Differential Diagnosis of Congenital Lactic Acidosis by Debray, Francois-Guillaume, Mitchell, Grant A, Allard, Pierre, Robinson, Brian H, Hanley, James A, Lambert, Marie

“…Although the blood lactate-to-pyruvate (L:P) molar ratio is used to distinguish between pyruvate dehydrogenase deficiency (PDH-D) and other causes of…”
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Mitochondrial citrate synthase crystals: Novel finding in Sengers syndrome caused by acylglycerol kinase (AGK) mutations by Siriwardena, Komudi, MacKay, Nevena, Levandovskiy, Valeriy, Blaser, Susan, Raiman, Julian, Kantor, Paul F., Ackerley, Cameron, Robinson, Brian H., Schulze, Andreas, Cameron, Jessie M.

“…We report on two families with Sengers syndrome and mutations in the acylglycerol kinase gene (AGK). In the first family, two brothers presented with vascular…”
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Human Complex I deficiency: Clinical spectrum and involvement of oxygen free radicals in the pathogenicity of the defect by Robinson, Brian H

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Control of oxygen free radical formation from mitochondrial complex I: roles for protein kinase A and pyruvate dehydrogenase kinase by Raha, Sandeep, Myint, A.Tomoko, Johnstone, Leslie, Robinson, Brian H

“…Human NADH CoQ oxidoreductase is composed of a total of 43 subunits and has been demonstrated to be a major site for the production of superoxide by…”
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Synthesis, Structure, and Redox Chemistry of Ethenyl and Ethynyl Ferrocene Polyaromatic Dyads by Cuffe, Laurence, Hudson, Richard D. A., Gallagher, John F., Jennings, Sarah, McAdam, C. John, Connelly, Rosamond B. T., Manning, Anthony R., Robinson, Brian. H., Simpson, Jim

“…A series of ferrocenyl−arene dyads, Fc−C⋮C−Ar, trans-Fc−CHCH−Ar, and Fc−CHCH−CHCH−Ar (Ar = phenyl, 1-naphthyl, 2-naphthyl, 9-phenanthryl, 9-anthryl,…”
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Thermodynamic and kinetic stability. Properties of micelles and vesicles formed by the decanoic acid/decanoate system by Morigaki, Kenichi, Walde, Peter, Misran, Misni, Robinson, Brian H

“…The dialysis of micellar and vesicular systems, through a cellulose acetate membrane permeable only to monomer surfactant, has been investigated for the system…”
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Micellization of Hydrocarbon Surfactants in Supercritical Carbon Dioxide by Eastoe, Julian, Paul, Alison, Nave, Sandrine, Steytler, David C, Robinson, Brian H, Rumsey, Emily, Thorpe, Matthew, Heenan, Richard. K

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Formation and Morphology of Calcium Sulfate Nanoparticles and Nanowires in Water-in-Oil Microemulsions by Rees, Gareth D, Evans-Gowing, Richard, Hammond, Stephen J, Robinson, Brian H

“…Nanosized insoluble metal sulfate aggregates, predominantly CaSO4, have been prepared in a variety of water-in-oil (w/o) microemulsions stabilized by either…”
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Rolandic Mitochondrial Encephalomyelopathy and MT-ND3 Mutations by Werner, Klaus G.E., MD, PhD, Morel, Chantal F., MD, Kirton, Adam, MD, Benseler, Susanne M., MD, Shoffner, John M., MD, Addis, Jane B.L., BSc, Robinson, Brian H., PhD, Burrowes, Delilah M., MD, Blaser, Susan I., MD, Epstein, Leon G., MD, Feigenbaum, Annette S.J., MD

“…Mitochondrial encephalopathies may be caused by mutations in the respiratory chain complex I subunit genes. Described here are the cases of two pediatric…”
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Automated Spectrophotometric Analysis of Mitochondrial Respiratory Chain Complex Enzyme Activities in Cultured Skin Fibroblasts by Kramer, Karen A, Oglesbee, Devin, Hartman, Stacy J, Huey, Joe, Anderson, Bambi, Magera, Mark J, Matern, Dietrich, Rinaldo, Piero, Robinson, Brian H, Cameron, Jessie M, Hahn, Si Houn

“…Mitochondrial respiratory chain complex (RCC) disorders may occur as commonly as 1 in 8500 individuals. Because of the great variability of phenotypic…”
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Slc25a13-Knockout Mice Harbor Metabolic Deficits but Fail To Display Hallmarks of Adult-Onset Type II Citrullinemia by Sinasac, David S., Moriyama, Mitsuaki, Jalil, M. Abdul, Begum, Laila, Li, Meng Xian, Iijima, Mikio, Horiuchi, Masahisa, Robinson, Brian H., Kobayashi, Keiko, Saheki, Takeyori, Tsui, Lap-Chee

“…Article Usage Stats Services MCB Citing Articles Google Scholar PubMed Related Content Social Bookmarking CiteULike Delicious Digg Facebook Google+ Mendeley…”
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Mitochondria, oxygen free radicals, disease and ageing by Raha, Sandeep, Robinson, Brian H

“…Superoxide is generated by the mitochondrial respiratory chain. The transformation of this superoxide into hydrogen peroxide and, under certain conditions,…”
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