A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy von Ogilvie, Isla, Kennaway, Nancy G, Shoubridge, Eric A

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Mutations in COX15 Produce a Defect in the Mitochondrial Heme Biosynthetic Pathway, Causing Early-Onset Fatal Hypertrophic Cardiomyopathy von Antonicka, Hana, Mattman, Andre, Carlson, Christopher G., Glerum, D. Moira, Hoffbuhr, Kristen C., Leary, Scot C., Kennaway, Nancy G., Shoubridge, Eric A.

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Identification and Characterization of a Common Set of Complex I Assembly Intermediates in Mitochondria from Patients with Complex I Deficiency von Antonicka, Hana, Ogilvie, Isla, Taivassalo, Tanja, Anitori, Roberto P., Haller, Ronald G., Vissing, John, Kennaway, Nancy G., Shoubridge, Eric A.

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Aerobic conditioning in patients with mitochondrial myopathies: Physiological, biochemical, and genetic effects von Taivassalo, Tanja, Shoubridge, Eric A., Chen, Jacqueline, Kennaway, Nancy G., DiMauro, Salvatore, Arnold, Douglas L., Haller, Ronald G.

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Pharmacological correction of neonatal lethal hepatic dysfunction in a murine model of hereditary tyrosinaemia type I von Grompe, Markus, Lindstedt, Sven, Al-Dhalimy, Muhsen, Kennaway, Nancy G, Papaconstantinou, John, Torres-Ramos, Carlos A, Ou, Ching-Nau, Finegold, Milton

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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 von Antonicka, Hana, Leary, Scot C., Guercin, Guy-Hellen, Agar, Jeffrey N., Horvath, Rita, Kennaway, Nancy G., Harding, Cary O., Jaksch, Michaela, Shoubridge, Eric A.

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The molecular basis for tissue specificity of the oxidative phosphorylation deficiencies in patients with mutations in the mitochondrial translation factor EFG1 von Antonicka, Hana, Sasarman, Florin, Kennaway, Nancy G., Shoubridge, Eric A.

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Loss of fumarylacetoacetate hydrolase is responsible for the neonatal hepatic dysfunction phenotype of lethal albino mice von GROMPE, M, MUHSEN AL-DHALIMY, FINEGOLD, M, CHING-NAN OU, BURLINGAME, T, KENNAWAY, N. G, SORIANO, P

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Mitochondrial Encephalomyopathy and Complex III Deficiency Associated with a Stop-Codon Mutation in the Cytochrome b Gene von Keightley, J. Andrew, Anitori, Roberto, Burton, Miriam D., Quan, Franklin, Buist, Neil R.M., Kennaway, Nancy G.

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Size and settling velocity of suspended flocs during a Phaeocystis bloom in the tidally stirred Irish Sea, NW European shelf von Jago, CF, Kennaway, GM, Novarino, G, Jones, SE

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A microdeletion in cytochrome c oxidase (COX) subunit III associated with COX deficiency and recurrent myoglobinuria von Keightley, J. Andrew, Hoffbuhr, Kristen C, Burton, Miriam D, Salas, Virginia M, Johnston, Wendy S.W, Penn, Andrew M.W, Buist, Neil R.M, Kennaway, Nancy G

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Remarkable improvement in adult Leigh syndrome with partial cytochrome c oxidase deficiency von GOLDENBERG, P. C, STEINER, R. D, MERKENS, L. S, DUNAWAY, T, EGAN, R. A, ZIMMERMAN, E. A, NESBIT, G, ROBINSON, B, KENNAWAY, N. G

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Structural origin of the green iridescence on the chelicerae of the red-backed jumping spider, Phidippus johnsoni (Salticidae: Araneae) von Ingram, A.L., Deparis, O., Boulenguez, J., Kennaway, G., Berthier, S., Parker, A.R.

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Mutations in the mitochondrial tRNA Ser(AGY) gene are associated with deafness, retinal degeneration, myopathy and epilepsy von Tuppen, Helen A L, Naess, Karin, Kennaway, Nancy G, Al-Dosary, Mazhor, Lesko, Nicole, Yarham, John W, Bruhn, Helene, Wibom, Rolf, Nennesmo, Inger, Weleber, Richard G, Blakely, Emma L, Taylor, Robert W, McFarland, Robert

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A Pathogenic 15-Base Pair Deletion in Mitochondrial DNA-encoded Cytochrome c Oxidase Subunit III Results in the Absence of Functional Cytochrome c Oxidase von Hoffbuhr, Kristen C., Davidson, Edgar, Filiano, Beth A., Davidson, Mercy, Kennaway, Nancy G., King, Michael P.

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Contrasting phenotypes in three patients with novel mutations in mitochondrial tRNA genes von Anitori, Roberto, Manning, Kara, Quan, Franklin, Weleber, Richard G., Buist, Neil R.M., Shoubridge, Eric A., Kennaway, Nancy G.

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Mutations in the mitochondrial tRNASer(AGY) gene are associated with deafness, retinal degeneration, myopathy and epilepsy von Tuppen, Helen AL, Naess, Karin, Kennaway, Nancy G, Al-Dosary, Mazhor, Lesko, Nicole, Yarham, John W, Bruhn, Helene, Wibom, Rolf, Nennesmo, Inger, Weleber, Richard G, Blakely, Emma L, Taylor, Robert W, McFarland, Robert

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Mitochondrial myopathy with succinate dehydrogenase and aconitase deficiency : abnormalities of several iron-sulfur proteins von HALL, R. E, HENRIKSSON, K. G, LEWIS, S. F, HALLER, R. G, KENNAWAY, N. G

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Zn isotopes as a new tracer of metal micronutrient usage in the oceans von Vance, D., Archer, C., Bermin, J., Kennaway, G., Cox, E.J., Statham, P.J., Lohan, M.C., Ellwood, M.J.

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Diabetes and mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (melas) : Radiolabeled polymerase chain reaction is necessary for accurate detection of l... von SMITH, M. L, HUA, X.-Y, MARSDEN, D. L, LIU, D, KENNAWAY, N. G, NGO, K.-Y, HAAS, R. H

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