Search Results - "Heales, S J"

Development of pharmacological strategies for mitochondrial disorders by Kanabus, M, Heales, S J, Rahman, S

“…Mitochondrial diseases are an unusually genetically and phenotypically heterogeneous group of disorders, which are extremely challenging to treat. Currently,…”
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Accumulation of Krebs cycle intermediates and over-expression of HIF1α in tumours which result from germline FH and SDH mutations by Pollard, P.J., Brière, J.J., Alam, N.A., Barwell, J., Barclay, E., Wortham, N.C., Hunt, T., Mitchell, M., Olpin, S., Moat, S.J., Hargreaves, I.P., Heales, S.J., Chung, Y.L., Griffiths, J.R., Dalgleish, A., McGrath, J.A., Gleeson, M.J., Hodgson, S.V., Poulsom, R., Rustin, P., Tomlinson, I.P.M.

“…The nuclear-encoded Krebs cycle enzymes, fumarate hydratase (FH) and succinate dehydrogenase (SDHB, -C and -D), act as tumour suppressors. Germline mutations…”
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Clinical Features and Pharmacotherapy of Childhood Monoamine Neurotransmitter Disorders by Ng, J., Heales, S. J. R., Kurian, M. A.

“…Childhood neurotransmitter disorders are increasingly recognised as an expanding group of inherited neurometabolic syndromes. They are caused by disturbance in…”
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Altered skeletal muscle insulin signaling and mitochondrial complex II-III linked activity in adult offspring of obese mice by Shelley, Piran, Martin-Gronert, Malgorzata S, Rowlerson, Anthea, Poston, Lucilla, Heales, S J R, Hargreaves, Iain P, McConnell, Josie M, Ozanne, Susan E, Fernandez-Twinn, Denise S

“…We recently reported insulin resistance in adult offspring of obese C57BL/6J mice. We have now evaluated whether parameters of skeletal muscle structure and…”
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Differential effect of nitric oxide on glutathione metabolism and mitochondrial function in astrocytes and neurones: implications for neuroprotection/neurodegeneration? by Gegg, M. E., Beltran, B., Salas‐Pino, S., Bolanos, J. P., Clark, J. B., Moncada, S., Heales, S. J. R.

“…Primary culture rat astrocytes exposed to the long acting nitric oxide donor (Z)‐1‐[2‐aminoethyl)‐N‐(2‐ammonioethyl)amino]diazen‐1‐ium‐1,2‐diolate (DETA‐NO)…”
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Glutathione deficiency in patients with mitochondrial disease: Implications for pathogenesis and treatment by Hargreaves, I. P., Sheena, Y., Land, J. M., Heales, S. J. R.

“…Summary Glutathione (GSH) is a key intracellular antioxidant. With regard to mitochondrial function, loss of GSH is associated with impairment of the electron…”
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Mitochondrial cytochrome c release: a factor to consider in mitochondrial disease? by Oppenheim, M. L. S., Hargreaves, I. P, Pope, S., Land, J. M., Heales, S. J. R.

“…Summary The pathogenesis of mitochondrial disorders has largely focused on the impairment of cellular energy metabolism. However, mitochondrial dysfunction has…”
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Tetrahydrobiopterin availability, nitric oxide metabolism and glutathione status in the hph‐1 mouse; implications for the pathogenesis and treatment of tetrahydrobiopterin deficiency states by Lam, A. A. J., Hyland, K., Heales, S. J. R.

“…Tetrahydrobiopterin (BH4) is an essential cofactor for all isoforms of nitric oxide synthase. While it is well established that BH4 deficiency states are…”
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Nitric oxide-mediated inhibition of the mitochondrial respiratory chain in cultured astrocytes by Bolaños, J P, Peuchen, S, Heales, S J, Land, J M, Clark, J B

“…The Ca(2+)-independent form of nitric oxide synthase was induced in rat neonatal astrocytes in primary culture by incubation with lipopolysaccharide (1…”
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Adaptation of the nitrate reductase and Griess reaction methods for the measurement of serum nitrate plus nitrite levels by Giovannoni, G, Land, J M, Keir, G, Thompson, E J, Heales, S J

“…Nitrite and nitrate determinations in biological fluids are increasingly being used as markers of nitric oxide production. We have modified a nitrate reductase…”
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Nitric oxide-dependent damage to neuronal mitochondria involves the NMDA receptor by Stewart, V. C., Heslegrave, A. J., Brown, G. C., Clark, J. B., Heales, S. J. R.

“…Cytokine‐stimulated astrocytes produce nitric oxide, which can inhibit components of the mitochondrial respiratory chain. We have previously demonstrated that…”
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Spectrum of movement disorders and neurotransmitter abnormalities in paediatric POLG disease by Papandreou, A., Rahman, S., Fratter, C., Ng, J., Meyer, E., Carr, L. J., Champion, M., Clarke, A., Gissen, P., Hemingway, C., Hussain, N., Jayawant, S., King, M. D., Lynch, B. J., Mewasingh, L., Patel, J., Prabhakar, P., Neergheen, V., Pope, S., Heales, S. J. R., Poulton, J., Kurian, Manju A.

“…Objectives To describe the spectrum of movement disorders and cerebrospinal fluid (CSF) neurotransmitter profiles in paediatric patients with POLG disease…”
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Effects of 1-methyl-4-phenylpyridinium on isolated rat brain mitochondria: evidence for a primary involvement of energy depletion by Bates, T E, Heales, S J, Davies, S E, Boakye, P, Clark, J B

“…The effects of 1-methyl-4-phenylpyridinium (MPP+) on the oxygen consumption, ATP production, H2O2 production, and mitochondrial NADH-CoQ1 reductase (complex I)…”
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Correction to: Spectrum of movement disorders and neurotransmitter abnormalities in paediatric POLG disease by Papandreou, A., Rahman, S., Fratter, C., Ng, J., Meyer, E., Carr, L. J., Champion, M., Clarke, A., Gissen, P., Hemingway, C., Hussain, N., Jayawant, S., King, M. D., Lynch, B. J., Mewasingh, L., Patel, J., Prabhakar, P., Neergheen, V., Pope, S., Heales, S. J. R., Poulton, J., Kurian, Manju A.

“…Due to a typesetting error the wrong Table 2 was used. The correct Table 2 is shown here:…”
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Diagnostic Value of Succinate Ubiquinone Reductase Activity in the Identification of Patients with Mitochondrial DNA Depletion by Hargreaves, I. P., Rahman, S., Guthrie, P., Taanman, J‐W., Leonard, J. V., Land, J. M., Heales, S. J. R.

“…Mitochondrial DNA (mtDNA) depletion syndrome (McKusick 251880) is characterized by a progressive quantitative loss of mtDNA resulting in severe mitochondrial…”
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Nitric Oxide‐Mediated Mitochondrial Damage in the Brain: Mechanisms and Implications for Neurodegenerative Diseases by Bolaños, Juan P., Almeida, Angeles, Stewart, Victoria, Peuchen, Stephan, Land, John M., Clark, John B., Heales, Simon J. R.

“…: Within the CNS and under normal conditions, nitric oxide (•NO) appears to be an important physiological signalling molecule. Its ability to increase cyclic…”
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Mitochondrial respiratory chain defects are not accompanied by an increase in the activities of lactate dehydrogenase or manganese superoxide dismutase in paediatric skeletal muscle biopsies by Hargreaves, I. P., Heales, S. J. R., Land, J. M.

“…Both the activity of lactate dehydrogenase (LDH) and the quantity of manganese superoxide dismutase (MnSOD) protein have been reported to be increased in…”
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Effect of peroxynitrite on the mitochondrial respiratory chain: differential susceptibility of neurones and astrocytes in primary culture by Bolaños, J P, Heales, S J, Land, J M, Clark, J B

“…The effect of the neurotoxic nitric oxide derivative, the peroxynitrite anion (ONOO-), on the activity of the mitochondrial respiratory chain complexes in…”
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Primary pyruvate dehydrogenase E3 binding protein deficiency with mild hyperlactataemia and hyperalaninaemia by Hargreaves, I. P., Heales, S. J. R., Briddon, A., Lee, P. J., Hanna, M. G., Land, J. M.

“…A case of pyruvate dehydrogenase E3 binding protein deficiency is reported in a 24‐year‐old male with encephalomyopathy. Blood lactate was only minimally…”
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Co-culture of neurones with glutathione deficient astrocytes leads to increased neuronal susceptibility to nitric oxide and increased glutamate-cysteine ligase activity by Gegg, M.E., Clark, J.B., Heales, S.J.R.

“…The antioxidant glutathione (GSH) plays an important role in protecting the mitochondrial electron transport chain (ETC) from damage by oxidative stress in…”
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