Publications by authors named "Simon J R Heales"

57Publications

CoQ Deficient Endothelial Cell Culture Model for the Investigation of CoQ Blood-Brain Barrier Transport.

Authors:
Luke Wainwright Iain P Hargreaves Ana R Georgian Charles Turner R Neil Dalton N Joan Abbott Simon J R Heales Jane E Preston

J Clin Med 2020 Oct 10;9(10). Epub 2020 Oct 10.

School of Cancer and Pharmaceutical Sciences, King's College London, London SE1 9NH, UK.

View Article and Find Full Text PDF
October 2020

DNAJC6 Mutations Disrupt Dopamine Homeostasis in Juvenile Parkinsonism-Dystonia.

Authors:
Joanne Ng Elisenda Cortès-Saladelafont Lucia Abela Pichet Termsarasab Kshitij Mankad Sniya Sudhakar Kathleen M Gorman Simon J R Heales Simon Pope Lorenzo Biassoni Barbara Csányi John Cain Karl Rakshi Helen Coutts Sandeep Jayawant Rosalind Jefferson Deborah Hughes Àngels García-Cazorla Detelina Grozeva F Lucy Raymond Belén Pérez-Dueñas Christian De Goede Toni S Pearson Esther Meyer Manju A Kurian

Mov Disord 2020 08 30;35(8):1357-1368. Epub 2020 May 30.

Molecular Neurosciences, Developmental Neurosciences Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom.

View Article and Find Full Text PDF
August 2020

Inhibition of Mitochondrial Complex I Impairs Release of α-Galactosidase by Jurkat Cells.

Authors:
Jonathan R A Lambert Steven J Howe Ahad A Rahim Derek G Burke Simon J R Heales

Int J Mol Sci 2019 Sep 5;20(18). Epub 2019 Sep 5.

Enzyme Unit Great Ormond Street Hospital, London WC1N 3JH, UK.

View Article and Find Full Text PDF
September 2019

PDXK mutations cause polyneuropathy responsive to pyridoxal 5'-phosphate supplementation.

Authors:
Viorica Chelban Matthew P Wilson Jodi Warman Chardon Jana Vandrovcova M Natalia Zanetti Eleni Zamba-Papanicolaou Stephanie Efthymiou Simon Pope Maria R Conte Giancarlo Abis Yo-Tsen Liu Eloise Tribollet Nourelhoda A Haridy Juan A Botía Mina Ryten Paschalis Nicolaou Anna Minaidou Kyproula Christodoulou Kristin D Kernohan Alison Eaton Matthew Osmond Yoko Ito Pierre Bourque James E C Jepson Oscar Bello Fion Bremner Carla Cordivari Mary M Reilly Martha Foiani Amanda Heslegrave Henrik Zetterberg Simon J R Heales Nicholas W Wood James E Rothman Kym M Boycott Philippa B Mills Peter T Clayton Henry Houlden

Ann Neurol 2019 08 1;86(2):225-240. Epub 2019 Jul 1.

Children's Hospital of Eastern Ontario Research Institute, University of Ottawa, Ottawa, Ontario, Canada.

View Article and Find Full Text PDF
August 2019

Mechanisms of action for the medium-chain triglyceride ketogenic diet in neurological and metabolic disorders.

Authors:
Katrin Augustin Aziza Khabbush Sophie Williams Simon Eaton Michael Orford J Helen Cross Simon J R Heales Matthew C Walker Robin S B Williams

Lancet Neurol 2018 01 16;17(1):84-93. Epub 2017 Dec 16.

Centre for Biomedical Sciences, School of Biological Sciences, Royal Holloway University of London, Egham, UK. Electronic address:

View Article and Find Full Text PDF
January 2018

An LC-MS/MS-Based Method for the Quantification of Pyridox(am)ine 5'-Phosphate Oxidase Activity in Dried Blood Spots from Patients with Epilepsy.

Authors:
Matthew P Wilson Emma J Footitt Apostolos Papandreou Mari-Liis Uudelepp Ronit Pressler Danielle C Stevenson Camila Gabriel Mel McSweeney Matthew Baggot Derek Burke Tommy Stödberg Kate Riney Manuel Schiff Simon J R Heales Kevin A Mills Paul Gissen Peter T Clayton Philippa B Mills

Anal Chem 2017 09 17;89(17):8892-8900. Epub 2017 Aug 17.

Genetics and Genomic Medicine, UCL GOS Institute of Child Health , 30 Guilford Street, London WC1N 1EH, United Kingdom.

View Article and Find Full Text PDF
September 2017

Neuronal decanoic acid oxidation is markedly lower than that of octanoic acid: A mechanistic insight into the medium-chain triglyceride ketogenic diet.

Authors:
Aziza Khabbush Michael Orford Yi-Chen Tsai Tricia Rutherford Maura O'Donnell Simon Eaton Simon J R Heales

Epilepsia 2017 08 6;58(8):1423-1429. Epub 2017 Jul 6.

Genetics and Genomic Medicine, UCL Great Ormond Street Institute of Child Health, London, United Kingdom.

View Article and Find Full Text PDF
August 2017

An examination of biochemical parameters and their association with response to ketogenic dietary therapies.

Authors:
Natasha E Schoeler Gail Bell Alan Yuen Adam D Kapelner Simon J R Heales J Helen Cross Sanjay Sisodiya

Epilepsia 2017 05 3;58(5):893-900. Epub 2017 Apr 3.

Department of Clinical and Experimental Epilepsy, NIHR University College London Hospitals Biomedical Research Centre, UCL Institute of Neurology, London, United Kingdom.

View Article and Find Full Text PDF
May 2017

Inhibition of neuronal mitochondrial complex I or lysosomal glucocerebrosidase is associated with increased dopamine and serotonin turnover.

Authors:
Carmen de la Fuente Derek G Burke Simon Eaton Simon J R Heales

Neurochem Int 2017 Oct 24;109:94-100. Epub 2017 Feb 24.

ICH Centre for Translational Omics, Genetic and Genomic Medicine Development Bio & Cancer Programme, UCL GOS Institute of Child Health and Chemical Pathology, Great Ormond Street Hospital, London, WC1N 3JH, UK. Electronic address:

View Article and Find Full Text PDF
October 2017

The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome.

Authors:
Marta Kanabus Elisa Fassone Sean David Hughes Sara Farahi Bilooei Tricia Rutherford Maura O' Donnell Simon J R Heales Shamima Rahman

J Inherit Metab Dis 2016 05 14;39(3):415-426. Epub 2016 Apr 14.

Genetics and Genomic Medicine, UCL Institute of Child Health, 30 Guilford Street, London, WC1N 1EH, UK.

View Article and Find Full Text PDF
May 2016

The novel R347g pathogenic mutation of aromatic amino acid decarboxylase provides additional molecular insights into enzyme catalysis and deficiency.

Authors:
Riccardo Montioli Alessandro Paiardini Manju A Kurian Mirco Dindo Giada Rossignoli Simon J R Heales Simon Pope Carla Borri Voltattorni Mariarita Bertoldi

Biochim Biophys Acta 2016 Jun 17;1864(6):676-682. Epub 2016 Mar 17.

Department of Neurosciences, Biomedicine and Movement, University of Verona, Verona, Italy. Electronic address:

View Article and Find Full Text PDF
June 2016

Drug-Induced Mitochondrial Toxicity.

Authors:
Iain P Hargreaves Mesfer Al Shahrani Luke Wainwright Simon J R Heales

Drug Saf 2016 07;39(7):661-74

Neurometabolic Unit. Box 105, National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK.

View Article and Find Full Text PDF
July 2016

Erratum to: Barth syndrome without tetralinoleoyl cardiolipin deficiency: a possible ameliorated phenotype.

Authors:
Ann Bowron Julie Honeychurch Maggie Williams Beverley Tsai-Goodman Nicol Clayton Lucy Jones Graham J Shortland Shakeel A Qureshi Simon J R Heales Colin G Steward

J Inherit Metab Dis 2016 Jan;39(1):151

NHS Barth Syndrome Service, Bristol Royal Hospital for Children, University Hospitals Bristol NHS Trust, Bristol, BS2 8BJ, UK.

View Article and Find Full Text PDF
January 2016

Biochemical diagnosis of coenzyme q10 deficiency.

Authors:
Delia Yubero Raquel Montero Rafael Artuch John M Land Simon J R Heales Iain P Hargreaves

Mol Syndromol 2014 Jul;5(3-4):147-55

Neurometabolic Unit, National Hospital for Neurology and Neurosurgery, London, UK.

View Article and Find Full Text PDF
July 2014

Barth syndrome without tetralinoleoyl cardiolipin deficiency: a possible ameliorated phenotype.

Authors:
Ann Bowron Julie Honeychurch Maggie Williams Beverley Tsai-Goodman Nicol Clayton Lucy Jones Graham J Shortland Shakeel A Qureshi Simon J R Heales Colin G Steward

J Inherit Metab Dis 2015 Mar 12;38(2):279-86. Epub 2014 Aug 12.

Department of Clinical Biochemistry, University Hospitals Bristol NHS Trust, Bristol, BS2 8HW, UK,

View Article and Find Full Text PDF
March 2015

Missense dopamine transporter mutations associate with adult parkinsonism and ADHD.

Authors:
Freja H Hansen Tina Skjørringe Saiqa Yasmeen Natascha V Arends Michelle A Sahai Kevin Erreger Thorvald F Andreassen Marion Holy Peter J Hamilton Viruna Neergheen Merete Karlsborg Amy H Newman Simon Pope Simon J R Heales Lars Friberg Ian Law Lars H Pinborg Harald H Sitte Claus Loland Lei Shi Harel Weinstein Aurelio Galli Lena E Hjermind Lisbeth B Møller Ulrik Gether

J Clin Invest 2014 Jul 9;124(7):3107-20. Epub 2014 Jun 9.

View Article and Find Full Text PDF
July 2014

The ketogenic diet component decanoic acid increases mitochondrial citrate synthase and complex I activity in neuronal cells.

Authors:
Sean David Hughes Marta Kanabus Glenn Anderson Iain P Hargreaves Tricia Rutherford Maura O'Donnell J Helen Cross Shamima Rahman Simon Eaton Simon J R Heales

J Neurochem 2014 May 25;129(3):426-33. Epub 2014 Jan 25.

Clinical and Molecular Genetics Unit, UCL Institute of Child Health, London, UK; Chemical Pathology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

View Article and Find Full Text PDF
May 2014

HIBCH mutations can cause Leigh-like disease with combined deficiency of multiple mitochondrial respiratory chain enzymes and pyruvate dehydrogenase.

Authors:
Sacha Ferdinandusse Hans R Waterham Simon J R Heales Garry K Brown Iain P Hargreaves Jan-Willem Taanman Roxana Gunny Lara Abulhoul Ronald J A Wanders Peter T Clayton James V Leonard Shamima Rahman

Orphanet J Rare Dis 2013 Dec 4;8:188. Epub 2013 Dec 4.

Metabolic Unit, Great Ormond Street Hospital, London, UK.

View Article and Find Full Text PDF
December 2013

Levels of 5-methyltetrahydrofolate and ascorbic acid in cerebrospinal fluid are correlated: implications for the accelerated degradation of folate by reactive oxygen species.

Authors:
Sophie-Beth Aylett Viruna Neergheen Iain P Hargreaves Simon Eaton John M Land Shamima Rahman Simon J R Heales

Neurochem Int 2013 Dec 15;63(8):750-5. Epub 2013 Oct 15.

Clinical and Molecular Genetics Unit, UCL Institute of Child Health, London, UK; Chemical Pathology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

View Article and Find Full Text PDF
December 2013

Coenzyme Q10 quantification in muscle, fibroblasts and cerebrospinal fluid by liquid chromatography/tandem mass spectrometry using a novel deuterated internal standard.

Authors:
Kate E C Duberley Iain P Hargreaves Korn-Anong Chaiwatanasirikul Simon J R Heales John M Land Shamima Rahman Kevin Mills Simon Eaton

Rapid Commun Mass Spectrom 2013 May;27(9):924-30

Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK.

View Article and Find Full Text PDF
May 2013

Dopamine but not l-dopa stimulates neural glutathione metabolism. Potential implications for Parkinson's and other dopamine deficiency states.

Authors:
George F G Allen Yasmin Ullah Iain P Hargreaves John M Land Simon J R Heales

Neurochem Int 2013 Apr 19;62(5):684-94. Epub 2012 Dec 19.

Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square House, Queen Square, London WC1N 3BG, UK.

View Article and Find Full Text PDF
April 2013

Diagnosis of Barth syndrome using a novel LC-MS/MS method for leukocyte cardiolipin analysis.

Authors:
Ann Bowron Robert Frost Vicki E C Powers Paul H Thomas Simon J R Heales Colin G Steward

J Inherit Metab Dis 2013 Sep 30;36(5):741-6. Epub 2012 Oct 30.

Department of Clinical Biochemistry, Bristol Royal Infirmary, University Hospitals Bristol NHS Foundation Trust, Bristol BS2 8HW, UK.

View Article and Find Full Text PDF
September 2013

Glucocerebrosidase deficiency in substantia nigra of parkinson disease brains.

Authors:
Matthew E Gegg Derek Burke Simon J R Heales J Mark Cooper John Hardy Nicholas W Wood Anthony H V Schapira

Ann Neurol 2012 Sep;72(3):455-63

Department of Clinical Neurosciences, University College London Institute of Neurology.

View Article and Find Full Text PDF
September 2012

An intragenic duplication in guanosine triphosphate cyclohydrolase-1 gene in a dopa-responsive dystonia family.

Authors:
Helen Ling James M Polke Mary G Sweeney Andrea Haworth Catherine A Sandford Simon J R Heales Nicholas W Wood Mary B Davis Andrew J Lees

Mov Disord 2011 Apr 1;26(5):905-9. Epub 2011 Feb 1.

Reta Lila Weston Institute of Neurological Studies, Institute of Neurology, University College London, London, United Kingdom.

View Article and Find Full Text PDF
April 2011

Depletion of glutathione does not affect electron transport chain complex activity in brain mitochondria: Implications for Parkinson disease and postmortem studies.

Authors:
Simon J R Heales Adrian Menzes Gavin P Davey

Free Radic Biol Med 2011 Apr 8;50(7):899-902. Epub 2010 Dec 8.

Clinical and Molecular Genetics Unit, University College London Institute of Child Health, London WC1N 1EH, UK.

View Article and Find Full Text PDF
April 2011

Hyperprolactinaemia and mental illness.

Authors:
John M Land Simon J R Heales

Ann Clin Biochem 2010 Nov 6;47(Pt 6):576. Epub 2010 Oct 6.

View Article and Find Full Text PDF
November 2010

Bezafibrate induced increase in mitochondrial electron transport chain complex IV activity in human astrocytoma cells: Implications for mitochondrial cytopathies and neurodegenerative diseases.

Authors:
Nicola Ioannou Iain P Hargreaves George Allen Kate Duberley John M Land Simon J R Heales

Biofactors 2010 Nov-Dec;36(6):468-73. Epub 2010 Sep 24.

Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, UK.

View Article and Find Full Text PDF
May 2011

Pyridoxal 5'-phosphate deficiency causes a loss of aromatic L-amino acid decarboxylase in patients and human neuroblastoma cells, implications for aromatic L-amino acid decarboxylase and vitamin B(6) deficiency states.

Authors:
George F G Allen Viruna Neergheen Marcus Oppenheim Julia C Fitzgerald Emma Footitt Keith Hyland Peter T Clayton John M Land Simon J R Heales

J Neurochem 2010 Jul 9;114(1):87-96. Epub 2010 Apr 9.

Department of Molecular Neuroscience, UCL Institute of Neurology, London WC1N 3BG, UK.

View Article and Find Full Text PDF
July 2010

Persistent mitochondrial damage by nitric oxide and its derivatives: neuropathological implications.

Authors:
Juan P Bolaños Simon J R Heales

Front Neuroenergetics 2010 3;2. Epub 2010 Feb 3.

Department of Biochemistry and Molecular Biology, Institute of Neurosciences of Castilla- Leon, University of Salamanca Salamanca, Spain.

View Article and Find Full Text PDF
July 2011

Consequences of long-term oral administration of the mitochondria-targeted antioxidant MitoQ to wild-type mice.

Authors:
Sergio Rodriguez-Cuenca Helena M Cochemé Angela Logan Irina Abakumova Tracy A Prime Claudia Rose Antonio Vidal-Puig Anthony C Smith David C Rubinsztein Ian M Fearnley Bruce A Jones Simon Pope Simon J R Heales Brian Y H Lam Sudeshna Guha Neogi Ian McFarlane Andrew M James Robin A J Smith Michael P Murphy

Free Radic Biol Med 2010 Jan 23;48(1):161-72. Epub 2009 Oct 23.

Department of Clinical Biochemistry, University of Cambridge Metabolic Research Laboratories, Institute of Metabolic Science, Cambridge CB2 0QQ, UK.

View Article and Find Full Text PDF
January 2010

Decreased ubiquinone availability and impaired mitochondrial cytochrome oxidase activity associated with statin treatment.

Authors:
Andrew J Duncan Iain P Hargreaves Maxwell S Damian John M Land Simon J R Heales

Toxicol Mech Methods 2009 Jan;19(1):44-50

Department of Molecular Neuroscience, UCL Institute of Neurology, London WC1N 1BG, UK.

View Article and Find Full Text PDF
January 2009

Homozygous loss-of-function mutations in the gene encoding the dopamine transporter are associated with infantile parkinsonism-dystonia.

Authors:
Manju A Kurian Juan Zhen Shu-Yuan Cheng Yan Li Santosh R Mordekar Philip Jardine Neil V Morgan Esther Meyer Louise Tee Shanaz Pasha Evangeline Wassmer Simon J R Heales Paul Gissen Maarten E A Reith Eamonn R Maher

J Clin Invest 2009 Jun 26;119(6):1595-603. Epub 2009 May 26.

Department of Medical and Molecular Genetics, University of Birmingham School of Medicine, Institute of Biomedical Research, Birmingham, United Kingdom.

View Article and Find Full Text PDF
June 2009

A new perspective on the treatment of aromatic L-amino acid decarboxylase deficiency.

Authors:
George F G Allen John M Land Simon J R Heales

Mol Genet Metab 2009 May 27;97(1):6-14. Epub 2009 Jan 27.

Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square House, Queen Square, London WC1N 3BG, UK.

View Article and Find Full Text PDF
May 2009

Inactivation of brain mitochondrial Lon protease by peroxynitrite precedes electron transport chain dysfunction.

Authors:
Lee Stanyer Wenche Jorgensen Osamu Hori John B Clark Simon J R Heales

Neurochem Int 2008 Sep 12;53(3-4):95-101. Epub 2008 Jun 12.

Department of Molecular Neuroscience, Institute of Neurology, University College London, London, UK.

View Article and Find Full Text PDF
September 2008

Oxidative stress and mitochondrial dysfunction in neurodegeneration; cardiolipin a critical target?

Authors:
Simon Pope John M Land Simon J R Heales

Biochim Biophys Acta 2008 Jul-Aug;1777(7-8):794-9. Epub 2008 Mar 29.

Neurometabolic Unit, National Hospital, UCLH Foundation Trust, UCL, Queen Square, London, WC1N 3BG, UK.

View Article and Find Full Text PDF
August 2008

Astrocytes protect against copper-catalysed loss of extracellular glutathione.

Authors:
Simon A S Pope Rosemary Milton Simon J R Heales

Neurochem Res 2008 Jul 12;33(7):1410-8. Epub 2008 Mar 12.

Division of Neurochemistry, Department of Molecular Neuroscience, Institute of Neurology, University College London, London WC1N 3BG, UK.

View Article and Find Full Text PDF
July 2008

Tetrahydrobiopterin availability in Parkinson's and Alzheimer's disease; potential pathogenic mechanisms.

Authors:
Richard H Foxton John M Land Simon J R Heales

Neurochem Res 2007 Apr-May;32(4-5):751-6. Epub 2006 Dec 27.

Department of Molecular Neuroscience, Institute of Neurology, Queen Square, London, WC1N 3BG, UK.

View Article and Find Full Text PDF
May 2007

Some observations upon biochemical causes of ataxia and a new disease entity ubiquinone, CoQ10 deficiency.

Authors:
John M Land Simon J R Heales Andrew J Duncan Iain P Hargreaves

Neurochem Res 2007 Apr-May;32(4-5):837-43. Epub 2006 Dec 21.

Neurometabolic Unit Box 105, National Hospital for Neurology & Neurosurgery, Queen Square, London, WC1N 3BG, UK.

View Article and Find Full Text PDF
May 2007

Mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration.

Authors:
Ference J Loupatty Peter T Clayton Jos P N Ruiter Rob Ofman Lodewijk Ijlst Garry K Brown David R Thorburn Robert A Harris Marinus Duran Carlos Desousa Steve Krywawych Simon J R Heales Ronald J A Wanders

Am J Hum Genet 2007 Jan 30;80(1):195-9. Epub 2006 Nov 30.

Department of Clinical Chemistry and Pediatrics, Emma Children's Hospital, Amsterdam, The Netherlands.

View Article and Find Full Text PDF
January 2007

Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells.

Authors:
Guido F Laube Vanita Shah Victoria C Stewart Iain P Hargreaves Mushfequr R Haq Simon J R Heales William G van't Hoff

Pediatr Nephrol 2006 Apr 1;21(4):503-9. Epub 2006 Mar 1.

Nephro-urology Unit, Institute of Child Health, University College London Medical School, London, UK.

View Article and Find Full Text PDF
April 2006

Determination of coenzyme Q10 status in blood mononuclear cells, skeletal muscle, and plasma by HPLC with di-propoxy-coenzyme Q10 as an internal standard.

Authors:
Andrew J Duncan Simon J R Heales Kevin Mills Simon Eaton John M Land Iain P Hargreaves

Clin Chem 2005 Dec;51(12):2380-2

Division of Neurochemistry, Institute of Neurology, London, UK.

View Article and Find Full Text PDF
December 2005

Induction of mitochondrial oxidative stress in astrocytes by nitric oxide precedes disruption of energy metabolism.

Authors:
Jake Jacobson Michael R Duchen John Hothersall John B Clark Simon J R Heales

J Neurochem 2005 Oct 16;95(2):388-95. Epub 2005 Aug 16.

Miriam Marks Division of Neurochemistry, Institute of Neurology, Department of Biology, University College London, London, UK.

View Article and Find Full Text PDF
October 2005

The effect of HMG-CoA reductase inhibitors on coenzyme Q10: possible biochemical/clinical implications.

Authors:
Iain P Hargreaves Andrew J Duncan Simon J R Heales John M Land

Drug Saf 2005 ;28(8):659-76

Neurometabolic Unit, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.

View Article and Find Full Text PDF
October 2005

Neonatal epileptic encephalopathy caused by mutations in the PNPO gene encoding pyridox(am)ine 5'-phosphate oxidase.

Authors:
Philippa B Mills Robert A H Surtees Michael P Champion Clare E Beesley Neil Dalton Peter J Scambler Simon J R Heales Anthony Briddon Irene Scheimberg Georg F Hoffmann Johannes Zschocke Peter T Clayton

Hum Mol Genet 2005 Apr 16;14(8):1077-86. Epub 2005 Mar 16.

Institute of Child Health, University College London with Great Ormond Street Hospital for Children, NHS Trust, London WC1N 1EH, UK.

View Article and Find Full Text PDF
April 2005

Nitric oxide and neurological disorders.

Authors:
Andrew J Duncan Simon J R Heales

Mol Aspects Med 2005 Feb-Apr;26(1-2):67-96. Epub 2005 Jan 24.

Division of Neurochemistry and Neurometabolic Unit, Institute of Neurology, Queen Square, London WC1N 3BG, United Kingdom.

View Article and Find Full Text PDF
June 2005

Neurodegeneration or neuroprotection: the pivotal role of astrocytes.

Authors:
Simon J R Heales Amanda A J Lam Andrew J Duncan John M Land

Neurochem Res 2004 Mar;29(3):513-9

Division of Neurochemistry, Institute of Neurology, University College London, Queen Square, London, United Kingdom.

View Article and Find Full Text PDF
March 2004

Mitochondrial disease: a historical, biochemical, and London perspective.

Authors:
John M Land John A Morgan-Hughes Iain Hargreaves Simon J R Heales

Neurochem Res 2004 Mar;29(3):483-91

Neurometabolic Unit, Box 105, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG.

View Article and Find Full Text PDF
March 2004

Nitric oxide-induced mitochondrial dysfunction: implications for neurodegeneration.

Authors:
Victoria C Stewart Simon J R Heales

Free Radic Biol Med 2003 Feb;34(3):287-303

Department of Molecular Pathogenesis, Division of Neurochemistry, Institute of Neurology, University College London, London, England.

View Article and Find Full Text PDF
February 2003

Oxidative phosphorylation: structure, function, and intermediary metabolism.

Authors:
Simon J R Heales Matthew E Gegg John B Clark

Int Rev Neurobiol 2002 ;53:25-56

Department of Neurochemistry, Clinical Biochemistry (Neurometabolic Unit), Institute of Neurology and National Hospital, London, WC1N 3BG, United Kingdom.

View Article and Find Full Text PDF
March 2003

Evaluation of the role of P-glycoprotein in inflammation induced blood-brain barrier damage.

Authors:
Kian H Tan Wendy M Purcell Simon J R Heales Julie D McLeod Roger D Hurst

Neuroreport 2002 Dec;13(18):2593-7

Department of Neurochemistry, Institute of Neurology, Univeristy College London, London WC1N 3BG, UK.

View Article and Find Full Text PDF
December 2002

Activated T cells mediate direct blood-brain barrier endothelial cell death and dysfunction.

Authors:
Kian H Tan Wendy M Purcell Simon J R Heales Julie D McLeod Roger D Hurst

Neuroreport 2002 Dec;13(18):2587-91

Department of Neurochemistry, Institute of Neurology, University College of London, London WC1N 3BG, UK.

View Article and Find Full Text PDF
December 2002

Further case of paroxysmal exercise-induced dystonia and some insights into pathogenesis.

Authors:
Michael H Barnett Paul R Jarman Simon J R Heales Kailash P Bhatia

Mov Disord 2002 Nov;17(6):1386-7

National Hospital for Neurology and Neurosurgery, Institute of Neurology, Queen Square, London, United Kingdom.

View Article and Find Full Text PDF
November 2002

Preservation of extracellular glutathione by an astrocyte derived factor with properties comparable to extracellular superoxide dismutase.

Authors:
Victoria C Stewart Rebecca Stone Matthew E Gegg Martyn A Sharpe Roger D Hurst John B Clark Simon J R Heales

J Neurochem 2002 Nov;83(4):984-91

Department of Molecular Pathogenesis, Division of Neurochemistry, UCL, Institute of Neurology, London, UK.

View Article and Find Full Text PDF
November 2002

Intracellular distribution of the fluorescent dye nonyl acridine orange responds to the mitochondrial membrane potential: implications for assays of cardiolipin and mitochondrial mass.

Authors:
Jake Jacobson Michael R Duchen Simon J R Heales

J Neurochem 2002 Jul;82(2):224-33

Department of Molecular Pathogenesis, Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, UK.

View Article and Find Full Text PDF
July 2002

Determination of glutamate-cysteine ligase (gamma-glutamylcysteine synthetase) activity by high-performance liquid chromatography and electrochemical detection.

Authors:
Matthew E Gegg John B Clark Simon J R Heales

Anal Biochem 2002 May;304(1):26-32

Department of Molecular Pathogenesis, Division of Neurochemistry, Institute of Neurology, UCL, Queen Square, London, WC1N 3BG, United Kingdom.

View Article and Find Full Text PDF
May 2002

Mitochondrial dysfunction associated with neuronal death following status epilepticus in rat.

Authors:
Hannah R Cock Xin Tong Iain P Hargreaves Simon J R Heales John B Clark Phillip N Patsalos Maria Thom Michael Groves Anthony H V Schapira Simon D Shorvon Matthew C Walker

Epilepsy Res 2002 Feb;48(3):157-68

Department of Clinical and Experimental Epilepsy, Institute of Neurology, University College London, Queen Square, WC1N 3BG, London, UK.

View Article and Find Full Text PDF
February 2002

Impairment of brain mitochondrial function by reactive nitrogen species: the role of glutathione in dictating susceptibility.

Authors:
Simon J R Heales Juan P Bolaños

Neurochem Int 2002 May;40(6):469-74

Department of Neurochemistry, Institute of Neurology and National Hospital, Queen Square, London WC1N 3BG, UK.

View Article and Find Full Text PDF
May 2002