Search Results - "Heales, S.J.R."

Effect of Coenzyme Q10 supplementation on mitochondrial electron transport chain activity and mitochondrial oxidative stress in Coenzyme Q10 deficient human neuronal cells by Duberley, K.E., Heales, S.J.R., Abramov, A.Y., Chalasani, A., Land, J.M., Rahman, S., Hargreaves, I.P.

“…Primary Coenzyme Q10 (CoQ10) deficiency is an autosomal recessive disorder with a heterogeneous clinical presentation. Common presenting features include both…”
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O6-1 Loss-of-function mutations in the gene encoding the dopamine transporter, SLC6A3, cause Infantile Parkinsonism Dystonia (IPD) by Kurian, M.A, Zhen, J, Cheng, S.-Y, Li, Y, Mordekar, S.R, Jardine, P, Morgan, N.V, Meyer, E, Tee, L, Pasha, S, Wassmer, E, Assmann, B, Heales, S.J.R, Gissen, P, Reith, M.E.A, Maher, E.R

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Inhibition of Tetrahydrobiopterin Synthesis Reducesin VivoNitric Oxide Production in Experimental Endotoxic Shock by Bune, A.J., Brand, M.P., Heales, S.J.R., Shergill, J.K., Cammack, R., Cook, H.T.

“…Nitric oxide synthesis requires the cofactor tetrahydrobiopterin. We have examined the effect on nitric oxide synthesis in experimental endotoxic shock of…”
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Increased urinary nitric oxide metabolites in patients with multiple sclerosis correlates with early and relapsing disease by Giovannoni, G., Silver, N.C., O'Riordan, J., Miller, R.F., Heales, S.J.R., Land, J.M., Elliot, M., Feldmann, M., Miller, D.H., Thompson, E.J.

“…Nitric oxide (NO) has been implicated in the immunopathogenesis of MS as a potential mediator of neuronal loss. To investigate the role of.NO in the…”
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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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Inhibition of mitochondrial complex IV leads to secondary loss complex II-III activity: implications for the pathogenesis and treatment of mitochondrial encephalomyopathies by Hargreaves, I P, Duncan, A J, Wu, L, Agrawal, A, Land, J M, Heales, S J R

“…Mitochondrial encephalomyopathies, arising from deficiencies of the electron transport chain (ETC) give rise to a wide clinical spectrum of presentation and…”
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N-acetylcysteine and Unverricht-Lundborg disease: Variable response and possible side effects by EDWARDS, M. J. J, HARGREAVES, I. P, HEALES, S. J. R, JONES, S. J, RAMACHANDRAN, V, BHATIA, K. P, SISODIYA, S

“…Serum glutathione levels were assessed in a patient with genetically proven Unverricht-Lundborg disease (ULD) before and during treatment with the antioxidant…”
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Alterations in expression of dopamine receptors and neuropeptides in the striatum of GTP cyclohydrolase-deficient mice by Zeng, B.-Y., Heales, S.J.R., Canevari, L., Rose, S., Jenner, P.

“…The hph-1 mice have defective tetrahydrobiopterin biosynthesis and share many neurochemical similarities with l-dopa-responsive dystonia (DRD) in humans. In…”
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Raised serum nitrate and nitrite levels in patients with multiple sclerosis by GIOVANNONI, G, HEALES, S. J. R, SILVER, N. C, O'RIORDAN, J, MILLER, R. F, LAND, J. M, CLARK, J. B, THOMPSON, E. J

“…Nitric oxide and its highly reactive derivative peroxynitrite have been implicated as non-specific inflammatory mediators of neuronal and oligodendrocyte…”
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Glutathione protects astrocytes from peroxynitrite-mediated mitochondrial damage: implications for neuronal/astrocytic trafficking and neurodegeneration by Barker, J E, Bolaños, J P, Land, J M, Clark, J B, Heales, S J

“…In this study we have examined the susceptibility of the mitochondrial respiratory chain of astrocytes and astrocytes depleted of glutathione to peroxynitrite…”
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Elevated cerebrospinal fluid and serum nitrate and nitrite levels in patients with central nervous system complications of HIV-1 infection: a correlation with blood-brain-barrier dysfunction by Giovannoni, G, Miller, R.F, Heales, S.J.R, Land, J.M, Harrison, M.J.G, Thompson, E.J

“…As nitric oxide (⋅NO) is hypothesised to play a role in the immunopathogenesis of neurological complications associated with inflammation, we compared levels…”
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Impairment of the nitric oxide/cyclic GMP pathway in cerebellar slices prepared from thehph-1 mouse by Brand, M.P., Briddon, A., Land, J.M., Clark, J.B., Heales, S.J.R.

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Postnatal development of the complexes of the electron transport chain in isolated rat brain mitochondria by Bates, T E, Almeida, A, Heales, S J, Clark, J B

“…The postnatal development of the complexes of the electron transport chain in isolated rat brain mitochondria were investigated. Nonsynaptosomal brain…”
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Impairment of the nitric oxide/cyclic GMP pathway in cerebellar slices prepared from the hph-1 mouse by Brand, M.P., Briddon, A., Land, J.M., Clark, J.B., Heales, S.J.R.

“…In this study, the effect of tetrahydrobiopterin deficiency on the nitric oxide/cGMP pathway has been investigated in cerebellar slices derived from the hph-1…”
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Diagnosis of medium chain acyl CoA dehydrogenase deficiency by measurement of cis-4-decenoic acid in dried blood spots by Heales, S J, Leonard, J V

“…Using gas chromatography-mass spectrometry (GC-MS), with selected ion monitoring, a method for measurement of cis-4-decenoate in dried blood spots was…”
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