Liverpool Skylilne

Pharmacy and Biomolecular Sciences

Dr Iain Hargreaves

Dr Iain Hargreaves

Telephone: 0151 231 2711

Publications

Journal article

Hargreaves I, Heaton RA, Mantle D. 2020. Disorders of Human Coenzyme Q10 Metabolism: An Overview International Journal of Molecular Sciences, 21 :6695-6695 DOI Author Url Publisher Url Public Url

Heaton RA, Heales S, Rahman K, Sexton D, Hargreaves I. 2020. The Effect of Cellular Coenzyme Q10 Deficiency on Lysosomal Acidification Journal of Clinical Medicine, DOI Author Url Public Url

Cotta A, Alston CL, Baptista-Junior S, Paim JF, Carvalho E, Navarro MM, Appleton M, Shiau Ng Y, Valicek J, da-Cunha-Junior AL, Lima MI, de la Rocque Ferreira A, Takata RI, Hargreaves I, Gorman GS, McFarland R, Pierre G, Taylor RW. 2020. Early‐onset coenzyme Q10 deficiency associated with ataxia and respiratory chain dysfunction due to novel pathogenic COQ8A variants, including a large intragenic deletion JIMD Reports, DOI Author Url Publisher Url Public Url

Ghosh R, Wood-Kaczmar A, Dobson L, Smith EJ, Sirinathsinghji EC, Kriston-Vizi J, Hargreaves I, Heaton R, Hermann F, Abramov AY, Lam AJ, Heales SJ, Ketteler R, Bates GP, Andre R, Tabrizi SJ. 2020. Expression of mutant exon 1 huntingtin fragments in human neural stem cells and neurons causes inclusion formation and mitochondrial dysfunction. The FASEB Journal, DOI Author Url Publisher Url Public Url

Turton N, Heaton R, Ismail F, Roberts S, Nelder S, Phillips S, Hargreaves I. 2020. The Effect of Organophosphate Exposure on Neuronal Cell Coenzyme Q10 Status. Neurochemical Research, DOI Author Url Publisher Url Public Url

Poole OV, Horga A, Hardy SA, Bugiardini E, Woodward CE, Hargreaves I, Merve A, Quinlivan R, Taylor RW, Hanna MG, Pitceathly RDS. 2020. Multisystem mitochondrial disease caused by a rare m.10038G>A mitochondrial tRNA Gly ( MT-TG ) variant Neurology Genetics, 6 DOI Publisher Url Public Url

Mantle D, Hargreaves IP. 2020. Coenzyme Q10 supplementation in non-alcoholic fatty liver disease: An overview Gastrointestinal Nursing, 18 :22-27 DOI

Ceresa C, Hutton S, Lajarin-Cuesta M, Heaton R, Hargreaves I, Fracchia L, Diaz M. 2020. Production of Mannosylerythritol Lipids (MELs) to be Used as Antimicrobial Agents against S. aureus ATCC 6538 Current Microbiology: an international journal, DOI Author Url Public Url

Bugiardin E, Bottani E, Marchet S, Poole OV, Beninca C, Horga A, Woodward C, Lam A, Hargreaves I, Chalasani A, Valerio A, Lamantea E, Venner K, Holton JL, Zeviani M, Houlden H, Quinlivan R, Lamperti C, Hanna MG, Pitceathly RDS. 2020. Expanding the molecular and phenotypic spectrum of truncating MT-ATP6 mutations Neurology Genetics, 6 DOI Publisher Url Public Url

Horga A, Woodward CE, Mills A, Pareés I, Hargreaves IP, Brown RM, Bugiardini E, Brooks T, Manole A, Remzova E, Rahman S, Reilly MM, Houlden H, Sweeney MG, Brown GK, Polke JM, Gago F, Parton MJ, Pitceathly RDS, Hanna MG. 2019. Differential phenotypic expression of a novel PDHA1 mutation in a female monozygotic twin pair Human Genetics, 138 :1313-1322 DOI Author Url Publisher Url Public Url

Ng X, Sadeghian M, Heales S, Hargreaves IP. 2019. Assessment of Mitochondrial Dysfunction in Experimental Autoimmune Encephalomyelitis (EAE) Models of Multiple Sclerosis INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES, 20 DOI Author Url Public Url

Hargreaves IP, Mantle D. 2019. Supplementation with selenium and coenzyme Q10 in critically ill patients BRITISH JOURNAL OF HOSPITAL MEDICINE, 80 :589-593 DOI Author Url

Keatley K, Stromei-Cleroux S, Wiltshire T, Rajala N, Burton G, Holt WV, Littlewood DTJ, Briscoe AG, Jung J, Ashkan K, Heales SJ, Pilkington GJ, Meunier B, McGeehan JE, Hargreaves IP, McGeehan RE. 2019. Integrated Approach Reveals Role of Mitochondrial Germ-Line Mutation F18L in Respiratory Chain, Oxidative Alterations, Drug Sensitivity, and Patient Prognosis in Glioblastoma International Journal of Molecular Sciences, 20 DOI Author Url Publisher Url Public Url

Heaton R, Millichap L, Saleem F, Gannon J, Begum G, Hargreaves IP. 2019. Current biochemical treatments of mitochondrial respiratory chain disorders Expert opinion on orphan drugs, 7 :277-285 DOI Author Url Publisher Url Public Url

Thueson E, Leadon DP, Heaton R, Hargreaves IP, Bayly WM. 2019. Effect of daily supplementation with ubiquinol on muscle coenzyme Q10 concentrations in Thoroughbred racehorses Comparative Exercise Physiology, 15 :219-226 DOI

Bugiardini E, Mitchell AL, Rosa ID, Horning-Do H-T, Pitmann AM, Poole OV, Holton JL, Shah S, Woodward C, Hargreaves I, Quinlivan R, Amunts A, Wiesner RJ, Houlden H, Holt IJ, Hanna MG, Pitceathly RDS, Spinazzola A. 2019. MRPS25 mutations impair mitochondrial translation and cause encephalomyopathy Human Molecular Genetics, DOI Author Url Publisher Url Public Url

Foti SC, Hargreaves I, Carrington S, Kiely AP, Houlden H, Holton JL. 2019. Cerebral mitochondrial electron transport chain dysfunction in multiple system atrophy and Parkinson's disease Scientific Reports, 9 DOI Author Url Publisher Url Public Url

Horga A, Bugiardini E, Manole A, Bremner F, Jaunmuktane Z, Dankwa L, Rebelo AP, Woodward CE, Hargreaves IP, Cortese A, Pittman AM, Brandner S, Polke JM, Pitceathly RDS, Züchner S, Hanna MG, Scherer SS, Houlden H, Reilly MM. 2019. Autosomal dominant optic atrophy and cataract "plus" phenotype including axonal neuropathy Neurology: Genetics, 5 DOI Author Url Public Url

Hargreaves I, Mantle D, Milford D. 2019. Chronic kidney disease and coenzyme Q10 supplementation Journal of Kidney Care, 4 :82-90 DOI Publisher Url Public Url

Mantle D, Hargreaves I. 2019. Coenzyme Q10 and Degenerative Disorders Affecting Longevity: An Overview Antioxidants, 8 DOI Author Url Publisher Url Public Url

Montero R, Yubero D, Salgado MC, Julieta Gonzalez M, Campistol J, del Mar O'Callaghan M, Pineda M, Delgadillo V, Maynou J, Fernandez G, Montoya J, Ruiz-Pesini E, Meavilla S, Neergheen V, Garcia-Cazorla A, Navas P, Hargreaves I, Artuch R. 2019. Plasma coenzyme Q(10) status is impaired in selected genetic conditions SCIENTIFIC REPORTS, 9 DOI Author Url Public Url

Hargreaves I, Mantle D. 2018. Vitamin K2 supplementation in haemodialysis patients Journal of Kidney Care, 3 :368-373 DOI Publisher Url Public Url

Mantle D, Hargreaves IP. 2018. Organophosphate poisoning and coenzyme Q10: an overview British Journal of Neuroscience Nursing, 14 :206-214 DOI

Hargreaves IP. 2018. Biochemical Assessment and Monitoring of Mitochondrial Disease JOURNAL OF CLINICAL MEDICINE, 7 DOI Author Url Public Url

Hargreaves I, Mody N, Land J, Heales S. 2018. Blood Mononuclear Cell Mitochondrial Respiratory Chain Complex IV Activity is Decreased in Multiple Sclerosis Patients: Effects of beta-Interferon Treatment Journal of Clinical Medicine, 7 DOI Author Url Publisher Url Public Url

Ahmed ST, Alston CL, Hopton S, He L, Hargreaves IP, Falkous G, Olahova M, McFarland R, Turnbull DM, Rocha MC, Taylor RW. 2017. Using a quantitative quadruple immunofluorescent assay to diagnose isolated mitochondrial Complex I deficiency Scientific Reports, 7 DOI Author Url Publisher Url Public Url

Al Shahrani M, Heales S, Hargreaves I, Orford M. 2017. Oxidative Stress: Mechanistic Insights into Inherited Mitochondrial Disorders and Parkinson's Disease JOURNAL OF CLINICAL MEDICINE, 6 DOI Author Url Public Url

Manole A, Jaunmuktane Z, Hargreaves IP, Ludtmann MHR, Salpietro V, Bello OD, Pope S, Pandraud A, Horga A, Scalco RS, Li A, Ashokkumar B, Lourenc¸o CM, Heales S, Horvath R, Chinnery PF, Toro C, Singleton AB, Jacques TS, Abramov AY, Muntoni F, Hanna MG, Reilly MM, Revesz T, Kullmann DM, Jepson JEC, Houlden H. 2017. Clinical, pathological and functional characterization of riboflavin-responsive neuropathy Brain, 140 :2820-2837 DOI Author Url Publisher Url Public Url

Ghose A, Taylor C, Howie A, Chalasani A, Hargreaves I, Milford D. 2017. Measurement of Respiratory Chain Enzyme Activity in Human Renal Biopsy Specimens Journal of Clinical Medicine, 6 :90-90 DOI Author Url Public Url

Stepien KM, Heaton R, Rankin S, Murphy A, Bentley J, Sexton D, Hargreaves I. 2017. Evidence of Oxidative Stress and Secondary Mitochondrial Dysfunction in Metabolic and Non-Metabolic Disorders Journal of Clinical Medicine, 6 DOI Author Url Publisher Url Public Url

Dyson A, Dal-Pizzol F, Sabbatini G, Lach AB, Galfo F, dos Santos Cardoso J, Pescador Mendonça B, Hargreaves I, Bollen Pinto B, Bromage DI, Martin JF, Moore KP, Feelisch M, Singer M. 2017. Ammonium tetrathiomolybdate following ischemia/reperfusion injury: Chemistry, pharmacology, and impact of a new class of sulfide donor in preclinical injury models Brohi K. PLoS Medicine, 14 :e1002310-e1002310 DOI Author Url Publisher Url Public Url

Bugiardini E, Poole OV, Manole A, Pittman AM, Horga A, Hargreaves I, Woodward CE, Sweeney MG, Holton JL, Taanman J-W, Plant GT, Poulton J, Zeviani M, Ghezzi D, Taylor J, Smith C, Fratter C, Kanikannan MA, Paramasivam A, Thangaraj K, Spinazzola A, Holt IJ, Houlden H, Hanna MG, Pitceathly RDS. 2017. Clinicopathologic and molecular spectrum of RNASEH1 -related mitochondrial disease Neurology Genetics, 3 :e149-e149 DOI Author Url Publisher Url Public Url

Neergheen V, Chalasani A, Wainwright L, Yubero D, Montero R, Artuch R, Hargreaves IP. 2017. Coenzyme Q10 in the treatment of mitochondrial disease Journal of Inborn Errors of Metabolism and Screening, DOI Publisher Url Public Url

Cornelius N, Wardman JH, Hargreaves IP, Neergheen V, Bie AS, Tümer Z, Nielsen JE, Nielsen TT. 2017. Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts: Effect of coenzyme Q10 supplementation on these parameters Mitochondrion, 34 :103-114 DOI Author Url Publisher Url Public Url

McKiernan P, Ball S, Santra S, Foster K, Fratter C, Poulton J, Craig K, McFarland R, Rahman S, Hargreaves I, Gupte G, Sharif K, Taylor RW. 2016. Incidence of Primary Mitochondrial Disease in Children Younger Than 2 Years Presenting With Acute Liver Failure Journal of Pediatric Gastroenterology and Nutrition, 63 :592-597 DOI Author Url Publisher Url Public Url

Tarry-Adkins JL, Fernandez-Twinn DS, Chen JH, Hargreaves IP, Neergheen V, Aiken CE, Ozanne SE. 2016. Poor maternal nutrition and accelerated postnatal growth induces an accelerated aging phenotype and oxidative stress in skeletal muscle of male rats. Disease Models and Mechanisms, 9 :1221-1229 DOI Author Url Publisher Url Public Url

Sadeghian M, Mastrolia V, Rezaei Haddad A, Mosley A, Mullali G, Schiza D, Sajic M, Hargreaves I, Heales S, Duchen MR, Smith KJ. 2016. Mitochondrial dysfunction is an important cause of neurological deficits in an inflammatory model of multiple sclerosis Scientific Reports, 6 DOI Author Url Publisher Url Public Url

Alston CL, Howard C, Olahova M, Hardy SA, He L, Murray PG, O'Sullivan S, Doherty G, Shield JPH, Hargreaves IP, Monavari AA, Knerr I, McCarthy P, Morris AAM, Thorburn DR, Prokisch H, Clayton PE, McFarland R, Hughes J, Crushell E, Taylor RW. 2016. A recurrent mitochondrial p.Trp22Arg NDUFB3 variant causes a distinctive facial appearance, short stature and a mild biochemical and clinical phenotype Journal of Medical Genetics, 53 :634-641 DOI Author Url Publisher Url Public Url

Abeti R, Parkinson MH, Hargreaves IP, Angelova PR, Sandi C, Pook MA, Giunti P, Abramov AY. 2016. 'Mitochondrial energy imbalance and lipid peroxidation cause cell death in Friedreich's ataxia' Cell Death and Disease, 7 DOI Author Url Publisher Url Public Url

Dombi E, Diot A, Morten K, Carver J, Lodge T, Fratter C, Ng YS, Liao C, Muir R, Blakely EL, Hargreaves I, Al-Dosary M, Sarkar G, Hickman SJ, Downes SM, Jayawant S, Yu-Wai-Man P, Taylor RW, Poulton J. 2016. The m.13051G > A mitochondrial DNA mutation results in variable neurology and activated mitophagy Neurology, 86 :1921-1923 DOI Author Url Publisher Url Public Url

Pavlidou E, Salpietro V, Phadke R, Hargreaves IP, Batten L, McElreavy K, Pitt M, Mankad K, Wilson C, Cutrupi MC, Ruggieri M, McCormick D, Saggar A, Kinali M. 2016. Pontocerebellar hypoplasia type 2D and optic nerve atrophy further expand the spectrum associated with selenoprotein biosynthesis deficiency European Journal of Paediatric Neurology, 20 :483-488 DOI Author Url

Hargreaves IP, Al Shahrani M, Wainwright L, Heales SJR. 2016. Drug-Induced Mitochondrial Toxicity Drug Safety, 39 :661-674 DOI Author Url

Schottlaender LV, Bettencourt C, Kiely AP, Chalasani A, Neergheen V, Holton JL, Hargreaves I, Houlden H. 2016. Coenzyme Q10 Levels Are Decreased in the Cerebellum of Multiple-System Atrophy Patients PLoS One, 11 DOI Author Url Publisher Url Public Url

Tarry-Adkins JL, Fernandez-Twinn DS, Hargreaves IP, Neergheen V, Aiken CE, Martin-Gronert MS, McConnell JM, Ozanne SE. 2016. Coenzyme am prevents hepatic fibrosis, inflammation, and oxidative stress in a male rat model of poor maternal nutrition and accelerated postnatal growth American Journal of Clinical Nutrition, 103 :579-588 DOI Author Url Publisher Url Public Url

Yubero D, Montero R, Ramos M, Neergheen V, Navas P, Artuch R, Hargreaves I. 2015. Determination of urinary coenzyme Q(10) by HPLC with electrochemical detection: Reference values for a paediatric population BIOFACTORS, 41 :424-430 DOI Author Url

Ormazabal A, Casado M, Molero-Luis M, Montoya J, Rahman S, Aylett S-B, Hargreaves I, Heales S, Artuch R. 2015. Can folic acid have a role in mitochondrial disorders? DRUG DISCOVERY TODAY, 20 :1349-1354 DOI Author Url

Tarry-Adkins JL, Fernandez-Twinn DS, Madsen R, Chen J-H, Carpenter A, Hargreaves IP, McConnell JM, Ozanne SE. 2015. Coenzyme Q(10) Prevents Insulin Signaling Dysregulation and Inflammation Prior to Development of Insulin Resistance in Male Offspring of a Rat Model of Poor Maternal Nutrition and Accelerated Postnatal Growth ENDOCRINOLOGY, 156 :3528-3537 DOI Author Url

Alston CL, Ceccatelli Berti C, Blakely EL, Olahova M, He L, McMahon CJ, Olpin SE, Hargreaves IP, Nolli C, McFarland R, Goffrini P, O'Sullivan MJ, Taylor RW. 2015. A recessive homozygous p.Asp92Gly SDHD mutation causes prenatal cardiomyopathy and a severe mitochondrial complex II deficiency Human Genetics, 134 :869-879 DOI Author Url Publisher Url Public Url

Lax NZ, Alston CL, Schon K, Park S-M, Krishnakumar D, He L, Falkous G, Ogilvy-Stuart A, Lees C, King RH, Hargreaves IP, Brown GK, McFarland R, Dean AF, Taylor RW. 2015. Neuropathologic Characterization of Pontocerebellar Hypoplasia Type 6 Associated With Cardiomyopathy and Hydrops Fetalis and Severe Multisystem Respiratory Chain Deficiency due to Novel RARS2 Mutations Journal of Neuropathology and Experimental Neurology, 74 :688-703 DOI Author Url Publisher Url Public Url

Salpietro V, Phadke R, Saggar A, Hargreaves IP, Yates R, Fokoloros C, Mankad K, Hertecant J, Ruggieri M, McCormick D, Kinali M. 2015. Zellweger syndrome and secondary mitochondrial myopathy EUROPEAN JOURNAL OF PEDIATRICS, 174 :557-563 DOI Author Url

Lear PV, Gonzalez-Touceda D, Porteiro Couto B, Viano P, Guymer V, Remzova E, Tunn R, Chalasani A, Garcia-Caballero T, Hargreaves IP, Tynan PW, Christian HC, Nogueiras R, Parrington J, Dieguez C. 2015. Absence of Intracellular Ion Channels TPC1 and TPC2 Leads to Mature-Onset Obesity in Male Mice, Due to Impaired Lipid Availability for Thermogenesis in Brown Adipose Tissue ENDOCRINOLOGY, 156 :975-986 DOI Author Url

Tarry-Adkins JL, Fernandez-Twinn DS, Chen J-H, Hargreaves IP, Martin-Gronert MS, McConnell JM, Ozanne SE. 2014. Nutritional programming of coenzyme Q: potential for prevention and intervention? FASEB JOURNAL, 28 :5398-5405 DOI Author Url

Balreira A, Boczonadi V, Barca E, Pyle A, Bansagi B, Appleton M, Graham C, Hargreaves IP, Rasic VM, Lochmueller H, Griffin H, Taylor RW, Naini A, Chinnery PF, Hirano M, Quinzii CM, Horvath R. 2014. ANO10 mutations cause ataxia and coenzyme Q(10) deficiency Journal of Neurology, 261 :2192-2198 DOI Author Url Publisher Url Public Url

Baruteau J, Hargreaves I, Krywawych S, Chalasani A, Land JM, Davison JE, Kwok MK, Christov G, Karimova A, Ashworth M, Anderson G, Prunty H, Rahman S, Gruenewald S. 2014. Successful reversal of propionic acidaemia associated cardiomyopathy: Evidence for low myocardial coenzyme Q(10) status and secondary mitochondrial dysfunction as an underlying pathophysiological mechanism MITOCHONDRION, 17 :150-156 DOI Author Url

Duberley KE, Heales SJR, Abramov AY, Chalasani A, Land JM, Rahman S, Hargreaves IP. 2014. Effect of Coenzyme Q(10) supplementation on mitochondrial electron transport chain activity and mitochondrial oxidative stress in Coenzyme Q(10) deficient human neuronal cells INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY, 50 :60-63 DOI Author Url

Hughes SD, Kanabus M, Anderson G, Hargreaves IP, Rutherford T, Donnell MO, Cross JH, Rahman S, Eaton S, Heales SJR. 2014. The ketogenic diet component decanoic acid increases mitochondrial citrate synthase and complex I activity in neuronal cells JOURNAL OF NEUROCHEMISTRY, 129 :426-433 DOI Author Url

Dunn L, Allen GFG, Mamais A, Ling H, Li A, Duberley KE, Hargreaves IP, Pope S, Holton JL, Lees A, Heales SJ, Bandopadhyay R. 2014. Dysregulation of glucose metabolism is an early event in sporadic Parkinson's disease NEUROBIOLOGY OF AGING, 35 :1111-1115 DOI Author Url

Tucci A, Liu Y-T, Preza E, Pitceathly RDS, Chalasani A, Plagnol V, Land JM, Trabzuni D, Ryten M, Jaunmuktane Z, Reilly MM, Brandner S, Hargreaves I, Hardy J, Singleton AB, Abramov AY, Houlden H. 2014. Novel C12orf65 mutations in patients with axonal neuropathy and optic atrophy Journal of Neurology, Neurosurgery and Psychiatry, 85 :486-492 DOI Author Url Publisher Url Public Url

Liu Y-T, Hersheson J, Plagnol V, Fawcett K, Duberley KEC, Preza E, Hargreaves IP, Chalasani A, Laura M, Wood NW, Reilly MM, Houlden H. 2014. Autosomal-recessive cerebellar ataxia caused by a novel ADCK3 mutation that elongates the protein: clinical, genetic and biochemical characterisation Journal of Neurology, Neurosurgery and Psychiatry, 85 :493-498 DOI Author Url Publisher Url Public Url

Gold WA, Williamson SL, Kaur S, Hargreaves IP, Land JM, Pelka GJ, Tam PPL, Christodoulou J. 2014. Mitochondrial dysfunction in the skeletal muscle of a mouse model of Rett syndrome (RTT): Implications for the disease phenotype MITOCHONDRION, 15 :10-17 DOI Author Url

Uusimaa J, Evans J, Smith C, Butterworth A, Craig K, Ashley N, Liao C, Carver J, Diot A, Macleod L, Hargreaves I, Al-Hussaini A, Faqeih E, Asery A, Al Balwi M, Eyaid W, Al-Sunaid A, Kelly D, van Mourik I, Ball S, Jarvis J, Mulay A, Hadzic N, Samyn M, Baker A, Rahman S, Stewart H, Morris AAM, Seller A, Fratter C, Taylor RW, Poulton J. 2014. Clinical, biochemical, cellular and molecular characterization of mitochondrial DNA depletion syndrome due to novel mutations in the MPV17 gene European Journal of Human Genetics, 22 :184-191 DOI Author Url Publisher Url Public Url

Yubero D, Montero R, Artuch R, Land JM, Heales SJR, Hargreaves IP. 2014. Biochemical diagnosis of coenzyme Q10 deficiency Molecular Syndromology, 5 :147-155 DOI

Hargreaves IP. 2014. Coenzyme Q10 as a therapy for mitochondrial disease International Journal of Biochemistry and Cell Biology, 49 :105-111 DOI Author Url

Fitzgerald JC, Ugun-Klusek A, Allen G, De Girolamo LA, Hargreaves I, Ufer C, Abramov AY, Billett EE. 2014. Monoamine oxidase-A knockdown in human neuroblastoma cells reveals protection against mitochondrial toxins FASEB JOURNAL, 28 :218-229 DOI Author Url

Foley AR, Menezes MP, Pandraud A, Gonzalez MA, Al-Odaib A, Abrams AJ, Sugano K, Yonezawa A, Manzur AY, Burns J, Hughes I, McCullagh BG, Jungbluth H, Lim MJ, Lin J-P, Megarbane A, Urtizberea JA, Shah AH, Antony J, Webster R, Broomfield A, Ng J, Mathew AA, O'Byrne JJ, Forman E, Scoto M, Prasad M, O'Brien K, Olpin S, Oppenheim M, Hargreaves I, Land JM, Wang MX, Carpenter K, Horvath R, Straub V, Lek M, Gold W, Farrell MO, Brandner S, Phadke R, Matsubara K, McGarvey ML, Scherer SS, Baxter PS, King MD, Clayton P, Rahman S, Reilly MM, Ouvrier RA, Christodoulou J, Zuechner S, Muntoni F, Houlden H. 2014. Treatable childhood neuronopathy caused by mutations in riboflavin transporter RFVT2 Brain, 137 :44-56 DOI Author Url Publisher Url Public Url

Ferdinandusse S, Waterham HR, Heales SJR, Brown GK, Hargreaves IP, Taanman J-W, Gunny R, Abulhoul L, Wanders RJA, Clayton PT, Leonard JV, Rahman S. 2013. HIBCH mutations can cause Leigh-like disease with combined deficiency of multiple mitochondrial respiratory chain enzymes and pyruvate dehydrogenase Orphanet Journal of Rare Diseases, 188 DOI Author Url Publisher Url Public Url

Pitceathly RDS, Taanman J-W, Rahman S, Meunier B, Sadowski M, Cirak S, Hargreaves I, Land JM, Nanji T, Polke JM, Woodward CE, Sweeney MG, Solanki S, Foley R, Hurles ME, Stalker J, Blake J, Holton JL, Phadke R, Muntoni F, Reilly MM, Hanna MG. 2013. COX10 Mutations Resulting in Complex Multisystem Mitochondrial Disease That Remains Stable Into Adulthood JAMA NEUROLOGY, 70 :1556-1561 DOI Author Url

Aylett SB, Neergheen V, Hargreaves IP, Eaton S, Land JM, Rahman S, Heales SJR. 2013. Levels of 5-methyltetrahydrofolate and ascorbic acid in cerebrospinal fluid are correlated: Implications for the accelerated degradation of folate by reactive oxygen species Neurochemistry International, 63 :750-755 DOI Author Url

Tarry-Adkins JL, Blackmore HL, Martin-Gronert MS, Fernandez-Twinn DS, McConnell JM, Hargreaves IP, Giussani DA, Ozanne SE. 2013. Coenzyme Q(10) prevents accelerated cardiac aging in a rat model of poor maternal nutrition and accelerated postnatal growth MOLECULAR METABOLISM, 2 :480-490 DOI Author Url

Cornelius N, Byron C, Hargreaves I, Guerra PF, Furdek AK, Land J, Radford WW, Frerman F, Corydon TJ, Gregersen N, Olsen RKJ. 2013. Secondary coenzyme Q10 deficiency and oxidative stress in cultured fibroblasts from patients with riboflavin responsive multiple Acyl-CoA dehydrogenation deficiency HUMAN MOLECULAR GENETICS, 22 :3819-3827 DOI Author Url

Holmstrom KM, Baird L, Zhang Y, Hargreaves I, Chalasani A, Land JM, Stanyer L, Yamamoto M, Dinkova-Kostova AT, Abramov AY. 2013. Nrf2 impacts cellular bioenergetics by controlling substrate availability for mitochondrial respiration Biology Open, 2 :761-770 DOI Author Url Publisher Url Public Url

Pitceathly RDS, Rahman S, Wedatilake Y, Polke JM, Cirak S, Foley AR, Sailer A, Hurles ME, Stalker J, Hargreaves I, Woodward CE, Sweeney MG, Muntoni F, Houlden H, Taanman J-W, Hanna MG. 2013. NDUFA4 Mutations Underlie Dysfunction of a Cytochrome c Oxidase Subunit Linked to Human Neurological Disease (vol 3, pg 1795, 2013) CELL REPORTS, 4 :402-402 DOI Author Url

Turkes F, Murphy E, Land J, Demiray B, Duberley K, Briddon A, Hargreaves I. 2013. Assessment of mitochondrial electron transport chain function in a primary astrocyte cell model of hyperhomocystinaemia TOXICOLOGY MECHANISMS AND METHODS, 23 :459-463 DOI Author Url

Montero R, Grazina M, Lopez-Gallardo E, Montoya J, Briones P, Navarro-Sastre A, Land JM, Hargreaves IP, Artuch R, del Mar O'Callaghan M, Jou C, Jimenez C, Bujan N, Pineda M, Garcia-Cazorla A, Nascimento A, Perez-Duenas B, Ruiz-Pesini E, Fratter C, Salviati L, Simoes M, Mendes C, Santos MJ, Diogo L, Garcia P, Navas P. 2013. Coenzyme Q(10) deficiency in mitochondrial DNA depletion syndromes MITOCHONDRION, 13 :337-341 DOI Author Url

Osellame LD, Rahim AA, Hargreaves IP, Gegg ME, Richard-Londt A, Brandner S, Waddington SN, Schapira AHV, Duchen MR. 2013. Mitochondria and Quality Control Defects in a Mouse Model of Gaucher Disease-Links to Parkinson's Disease Cell Metabolism, 17 :941-953 DOI Author Url Publisher Url Public Url

Pitceathly RDS, Rahman S, Wedatilake Y, Polke JM, Cirak S, Foley AR, Sailer A, Hurles ME, Stalker J, Hargreaves I, Woodward CE, Sweeney MG, Muntoni F, Houlden H, Taanman J-W, Hanna MG. 2013. NDUFA4 Mutations Underlie Dysfunction of a Cytochrome c Oxidase Subunit Linked to Human Neurological Disease CELL REPORTS, 3 :1795-1805 DOI Author Url

Yao Z, Jones AWE, Fassone E, Sweeney MG, Lebiedzinska M, Suski JM, Wieckowski MR, Tajeddine N, Hargreaves IP, Yasukawa T, Tufo G, Brenner C, Kroemer G, Rahman S, Szabadkai G. 2013. PGC-1 beta mediates adaptive chemoresistance associated with mitochondrial DNA mutations ONCOGENE, 32 :2592-2600 DOI Author Url

Duberley KEC, Hargreaves IP, Chaiwatanasirikul K-A, Heales SJR, Land JM, Rahman S, Mills K, Eaton S. 2013. Coenzyme Q(10) quantification in muscle, fibroblasts and cerebrospinal fluid by liquid chromatography/tandem mass spectrometry using a novel deuterated internal standard RAPID COMMUNICATIONS IN MASS SPECTROMETRY, 27 :924-930 DOI Author Url

Allen GFG, Ullah Y, Hargreaves IP, Land JM, Heales SJR. 2013. Dopamine but not L-dopa stimulates neural glutathione metabolism. Potential implications for Parkinson's and other dopamine deficiency states NEUROCHEMISTRY INTERNATIONAL, 62 :684-694 DOI Author Url

Duberley KEC, Abramov AY, Chalasani A, Heales SJ, Rahman S, Hargreaves IP. 2013. Human neuronal coenzyme Q(10) deficiency results in global loss of mitochondrial respiratory chain activity, increased mitochondrial oxidative stress and reversal of ATP synthase activity: implications for pathogenesis and treatment JOURNAL OF INHERITED METABOLIC DISEASE, 36 :63-73 DOI Author Url

Tarry-Adkins JL, Martin-Gronert MS, Fernandez-Twinn DS, Hargreaves I, Alfaradhi MZ, Land JM, Aiken CE, Ozanne SE. 2013. Poor maternal nutrition followed by accelerated postnatal growth leads to alterations in DNA damage and repair, oxidative and nitrosative stress, and oxidative defense capacity in rat heart FASEB JOURNAL, 27 :379-390 DOI Author Url

Pitceathly RDS, Tomlinson SE, Hargreaves I, Bhardwaj N, Holton JL, Morrow JM, Evans J, Smith C, Fratter C, Woodward CE, Sweeney MG, Rahman S, Hanna MG. 2013. Distal myopathy with cachexia: an unrecognised phenotype caused by dominantly-inherited mitochondrial polymerase gamma mutations JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 84 :107-110 DOI Author Url

Pitceathly RDS, Murphy SM, Cottenie E, Chalasani A, Sweeney MG, Woodward C, Mudanohwo EE, Hargreaves I, Heales S, Land J, Holton JL, Houlden H, Blake J, Champion M, Flinter F, Robb SA, Page R, Palace J, Crowe C, Longman C, Lunn MP, Rahman S, Reilly MM, Hanna MG. 2012. Genetic dysfunction of MT-ATP6 causes axonal Charcot-Marie-Tooth disease NEUROLOGY, 79 :1145-1154 DOI Author Url

Damian MS, Hargreaves I. 2012. Ubiquinone in cardiac arrest: From bathroom to bedside RESUSCITATION, 83 :928-929 DOI Author Url

Ashrafian H, Czibik G, Bellahcene M, Aksentijević D, Smith AC, Mitchell SJ, Dodd MS, Kirwan J, Byrne JJ, Ludwig C, Isackson H, Yavari A, Støttrup NB, Contractor H, Cahill TJ, Sahgal N, Ball DR, Birkler RID, Hargreaves I, Tennant DA, Land J, Lygate CA, Johannsen M, Kharbanda RK, Neubauer S, Redwood C, De Cabo R, Ahmet I, Talan M, Günther UL, Robinson AJ, Viant MR, Pollard PJ, Tyler DJ, Watkins H. 2012. Fumarate is cardioprotective via activation of the Nrf2 antioxidant pathway Cell Metabolism, 15 :361-371 DOI Author Url

Fassone E, Taanman J-W, Hargreaves IP, Sebire NJ, Cleary MA, Burch M, Rahman S. 2011. Mutations in the mitochondrial complex I assembly factor NDUFAF1 cause fatal infantile hypertrophic cardiomyopathy JOURNAL OF MEDICAL GENETICS, 48 :691-697 DOI Author Url

Avis HJ, Hargreaves IP, Ruiter JPN, Land JM, Wanders RJ, Wijburg FA. 2011. Rosuvastatin Lowers Coenzyme Q10 Levels, but not Mitochondrial Adenosine Triphosphate Synthesis, in Children with Familial Hypercholesterolemia JOURNAL OF PEDIATRICS, 158 :458-462 DOI Author Url

Ioannou N, Hargreaves IP, Allen G, Duberley K, Land JM, Heales SJR. 2010. Bezafibrate induced increase in mitochondrial electron transport chain complex IV activity in human astrocytoma cells: Implications for mitochondrial cytopathies and neurodegenerative diseases BIOFACTORS, 36 :468-473 DOI Author Url

Ashrafian H, Docherty L, Leo V, Towlson C, Neilan M, Steeples V, Lygate CA, Hough T, Townsend S, Williams D, Wells S, Norris D, Glyn-Jones S, Land J, Barbaric I, Lalanne Z, Denny P, Szumska D, Bhattacharya S, Griffin JL, Hargreaves I, Fernandez-Fuentes N, Cheeseman M, Watkins H, Dear TN. 2010. A Mutation in the Mitochondrial Fission Gene Dnm1l Leads to Cardiomyopathy PLoS Genetics, 6 DOI Author Url Publisher Url Public Url

Alston CL, Bender A, Hargreaves IP, Mundy H, Deshpande C, Klopstock T, McFarland R, Horvath R, Taylor RW. 2010. The pathogenic m.3243A > T mitochondrial DNA mutation is associated with a variable neurological phenotype NEUROMUSCULAR DISORDERS, 20 :403-406 DOI Author Url

Trollet C, Anvar SY, Venema A, Hargreaves IP, Foster K, Vignaud A, Ferry A, Negroni E, Hourde C, Baraibar MA, 't Hoen PAC, Davies JE, Rubinsztein DC, Heales SJ, Mouly V, van der Maarel SM, Butler-Browne G, Raz V, Dickson G. 2010. Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres HUMAN MOLECULAR GENETICS, 19 :2191-2207 DOI Author Url

Alston CL, Morak M, Reid C, Hargreaves IP, Pope SAS, Land JM, Heales SJ, Horvath R, Mundy H, Taylor RW. 2010. A novel mitochondrial MTND5 frameshift mutation causing isolated complex I deficiency, renal failure and myopathy NEUROMUSCULAR DISORDERS, 20 :131-135 DOI Author Url

Shelley P, Martin-Gronert MS, Rowlerson A, Poston L, Heales SJR, Hargreaves IP, McConnell JM, Ozanne SE, Fernandez-Twinn DS. 2009. Altered skeletal muscle insulin signaling and mitochondrial complex II-III linked activity in adult offspring of obese mice AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY, 297 :R675-R681 DOI Author Url

Duncan AJ, Bitner-Glindzicz M, Meunier B, Costello H, Hargreaves IP, Lopez LC, Hirano M, Quinzii CM, Sadowski MI, Hardy J, Singleton A, Clayton PT, Rahman S. 2009. A Nonsense Mutation in COQ9 Causes Autosomal-Recessive Neonatal-Onset Primary Coenzyme Q(10) Deficiency: A Potentially Treatable Form of Mitochondrial Disease AMERICAN JOURNAL OF HUMAN GENETICS, 84 :558-566 DOI Author Url

Kyprianou N, Murphy E, Lee P, Hargreaves I. 2009. Assessment of mitochondrial respiratory chain function in hyperphenylalaninaemia JOURNAL OF INHERITED METABOLIC DISEASE, 32 :289-296 DOI Author Url

Oppenheim MLS, Hargreaves IP, Pope S, Land JM, Heales SJR. 2009. Mitochondrial cytochrome c release: a factor to consider in mitochondrial disease? JOURNAL OF INHERITED METABOLIC DISEASE, 32 :269-273 DOI Author Url

Duncan AJ, Hargreaves IP, Damian MS, Land JM, Heales SJR. 2009. Decreased Ubiquinone Availability and Impaired Mitochondrial Cytochrome Oxidase Activity Associated With Statin Treatment TOXICOLOGY MECHANISMS AND METHODS, 19 :44-50 DOI Author Url

Hargreaves IP, Lane A, Sleiman PMA. 2008. The coenzyme Q(10) status of the brain regions of Parkinson's disease patients NEUROSCIENCE LETTERS, 447 :17-19 DOI Author Url

Brooks KJ, Hargreaves IP, Bates TE. 2008. Protection of respiratory chain enzymes from ischaemic damage in adult rat brain slices NEUROCHEMICAL RESEARCH, 33 :1711-1716 DOI Author Url

Wood-Kaczmar A, Gandhi S, Yao Z, Abramov ASY, Miljan EA, Keen G, Stanyer L, Hargreaves I, Klupsch K, Deas E, Downward J, Mansfield L, Jat P, Taylor J, Heales S, Duchen MR, Latchman D, Tabrizi SJ, Wood NW. 2008. PINK1 Is Necessary for Long Term Survival and Mitochondrial Function in Human Dopaminergic Neurons PLoS One, 3 DOI Author Url Publisher Url Public Url

McKenzie M, Liolitsa D, Akinshina N, Campanella M, Sisodiya S, Hargreaves I, Nirmalananthan N, Sweeney MG, Abou-Sleiman PM, Wood NW, Hanna MG, Duchen MR. 2007. Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption JOURNAL OF BIOLOGICAL CHEMISTRY, 282 :36845-36852 DOI Author Url

Hargreaves IP, Duncan AJ, Wu L, Agrawal A, Land JM, Heales SJR. 2007. Inhibition of mitochondrial complex IV leads to secondary loss complex II-III activity: Implications for the pathogenesis and treatment of mitochondrial encephalomyopathies MITOCHONDRION, 7 :284-287 DOI Author Url

Hargreaves IP. 2007. Coenzyme Q10 in phenylketonuria and mevalonic aciduria Mitochondrion, 7 DOI Author Url

Rahman S, Hargreaves IP. 2007. Allogeneic stem cell transplantation corrects biochemical derangements in MNGIE NEUROLOGY, 68 :1872-1872 DOI Author Url

Land JM, Heales SJR, Duncan AJ, Hargreaves IP. 2007. Some observations upon biochemical causes of ataxia and a new disease entity ubiquinone, CoQ(10) deficiency NEUROCHEMICAL RESEARCH, 32 :837-843 DOI Author Url

Pagnamenta AT, Hargreaves IP, Duncan AJ, Taanman J-W, Heales SJ, Land JM, Bitner-Glindzicz M, Leonard JV, Rahman S. 2006. Phenotypic variability of mitochondrial disease caused by a nuclear mutation in complex II MOLECULAR GENETICS AND METABOLISM, 89 :214-221 DOI Author Url

Gandhi S, Muqit MMK, Stanyer L, Healy DG, Abou-Sleiman PM, Hargreaves I, Heales S, Ganguly M, Parsons L, Lees AJ, Latchman DS, Holton JL, Wood NW, Revesz T. 2006. PINK1 protein in normal human brain and Parkinson's disease BRAIN, 129 :1720-1731 DOI Author Url

Laube GF, Shah V, Stewart VC, Hargreaves IP, Haq MR, Heales SJR, van't Hoff WG. 2006. Glutathione depletion and increased apoptosis rate in human cystinotic proximal tubular cells PEDIATRIC NEPHROLOGY, 21 :503-509 DOI Author Url

Mordekar SR, Guthrie P, Bonham JR, Olpin SE, Hargreaves I, Baxter PS. 2006. The significance of reduced respiratory chain enzyme activities: Clinical, biochemical and radiological associations EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY, 10 :78-82 DOI Author Url

Duncan AJ, Heales SJR, Mills K, Eaton S, Land JM, Hargreaves IP. 2005. Determination of coenzyme Q10 status in blood mononuclear cells, skeletal muscle, and plasma by HPLC with di-propoxy-coenzyme Q10 as an internal standard Clinical Chemistry, 51 :2380-2382 DOI Author Url

Pollard PJ, Briere JJ, Alam NA, Barwell J, Barclay E, Wortham NC, Hunt T, Mitchell M, Olpin S, Moat SJ, Hargreaves IP, Heales SJ, Chung YL, Griffiths JR, Dalgleish A, McGrath JA, Gleeson MJ, Hodgson SV, Poulsom R, Rustin P, Tomlinson IPM. 2005. Accumulation of Krebs cycle intermediates and over-expression of HIF1 alpha in tumours which result from germline FH and SDH mutations HUMAN MOLECULAR GENETICS, 14 :2231-2239 DOI Author Url

Pulkes T, Liolitsa D, Wills AJ, Hargreaves I, Heales S, Hanna MG. 2005. Nonsense mutations in mitochondrial DNA associated with myalgia and exercise intolerance NEUROLOGY, 64 :1091-1092 DOI Author Url

Daley E, Wilkie D, Loesch A, Hargreaves IP, Kendall DA, Pilkington GJ, Bates TE. 2005. Chlorimipramine: A novel anticancer agent with a mitochondrial target BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 328 :623-632 DOI Author Url

Hargreaves IP, Sheena Y, Land JM, Heales SJR. 2005. Glutathione deficiency in patients with mitochondrial disease: Implications for pathogenesis and treatment JOURNAL OF INHERITED METABOLIC DISEASE, 28 :81-88 DOI Author Url

Hargreaves IP, Duncan AJ, Heales SJR, Land JM. 2005. The effect of HMG-CoA reductase inhibitors on coenzyme Q10 - Possible biochemical/clinical implications DRUG SAFETY, 28 :659-676 DOI Author Url

Martins LM, Morrison A, Klupsch K, Fedele V, Moisoi N, Teismann P, Abuin A, Grau E, Geppert M, Livi GP, Creasy CL, Martin A, Hargreaves I, Heales SJ, Okada H, Brandner S, Schulz JB, Mak T, Downward J. 2004. Neuroprotective role of the reaper-related serine protease HtrA2/Omi revealed by targeted deletion in mice Molecular and Cellular Biology, 24 :9848-9862 DOI Author Url

Land JM, Morgan-Hughes JA, Hargreaves I, Heales SJR. 2004. Mitochondrial disease: A historical, biochemical, and London perspective NEUROCHEMICAL RESEARCH, 29 :483-491 DOI Author Url

Hargreaves IP. 2003. Ubiquinone: Cholesterol's reclusive cousin Annals of Clinical Biochemistry, 40 :207-218 DOI Author Url

Hargreaves IP, Heales SJR, Briddon A, Lee PJ, Hanna MG, Land JM. 2003. Primary pyruvate dehydrogenase E3 binding protein deficiency with mild hyperlactataemia and hyperalaninaemia JOURNAL OF INHERITED METABOLIC DISEASE, 26 :505-506 DOI Author Url

Hargreaves IP. 2003. Coenzyme-Q(10) concentration in human phenylketonuria - Response JOURNAL OF INHERITED METABOLIC DISEASE, 26 :823-823 Author Url

Hargreaves IP, Heales SJR, Briddon A, Land JM, Lee PJ. 2002. Blood mononuclear cell coenzyme Q(10) concentration and mitochondrial respiratory chain succinate cytochrome-c reductase activity in phenylketonuric patients JOURNAL OF INHERITED METABOLIC DISEASE, 25 :673-679 DOI Author Url

Brooks KJ, Hargreaves I, Bhakoo K, Sellwood M, O'Brien F, Noone M, Sakata Y, Cady E, Wylezinska M, Thornton J, Ordidge R, Nguyen Q, Clemence M, Wyatt J, Bates TE. 2002. Delayed hypothermia prevents decreases in N-acetylaspartate and reduced glutathione in the cerebral cortex of the neonatal pig following transient hypoxia-ischaemia NEUROCHEMICAL RESEARCH, 27 :1599-1604 DOI Author Url

Edwards MJJ, Hargreaves IP, Heales SJR, Jones SJ, Ramachandran V, Bhatia KP, Sisodiya S. 2002. N-acetylcysteine and Unverricht-Lundborg disease - Variable response and possible side effects NEUROLOGY, 59 :1447-1449 DOI Author Url

Cifelli PM, Hargreaves I, Grunewald S. 2002. Cytochrome oxidase deficiency in Lowe syndrome JOURNAL OF INHERITED METABOLIC DISEASE, 25 :411-412 DOI Author Url

Brealey D, Brand M, Hargreaves I, Heales S, Land J, Smolenski R, Davies NA, Cooper CE, Singer M. 2002. Association between mitochondrial dysfunction and severity and outcome of septic shock LANCET, 360 :219-223 DOI Author Url

Hargreaves IP, Rahman S, Guthrie P, Taanman JW, Leonard JV, Land JM, Heales SJR. 2002. Diagnostic value of succinate ubiquinone reductase activity in the identification patients with mitochondrial DNA depletion Journal of Inherited Metabolic Disease, 25 :7-16 DOI Author Url

Hargreaves IP, Heales S. 2002. Statins and myopathy LANCET, 359 :711-712 DOI Author Url

Cock HR, Tong X, Hargreaves IP, Heales SJR, Clark JB, Patsalos PN, Thom M, Groves M, Schapira AHV, Shorvon SD, Walker MC. 2002. Mitochondrial dysfunction associated with neuronal death following status epilepticus in rat EPILEPSY RESEARCH, 48 :157-168 DOI Author Url

Hargreaves IP, Lee PJ, Briddon A. 2002. Homocysteine and cysteine-albumin binding in homocystinuria: assessment of cysteine status and implications for glutathione synthesis? AMINO ACIDS, 22 :109-118 DOI Author Url

Rahman S, Hargreaves I, Clayton P, Heales S. 2001. Neonatal presentation of coenzyme Q(10) deficiency JOURNAL OF PEDIATRICS, 139 :456-458 DOI Author Url

Brooks KJ, Hargreaves IP, Bates TE. 2000. Nitric-oxide-induced inhibition of mitochondrial complexes following aglycaemic hypoxia in neonatal cortical rat brain slices DEVELOPMENTAL NEUROSCIENCE, 22 :359-365 DOI Author Url

Hargreaves IP, Heales SJR, Land JM. 1999. 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 JOURNAL OF INHERITED METABOLIC DISEASE, 22 :925-931 DOI Author Url

Rahman S, Taanman JW, Cooper JM, Nelson I, Hargreaves I, Meunier B, Hanna MG, Garcia JJ, Capaldi RA, Lake BD, Leonard JV, Schapira AHV. 1999. A missense mutation of cytochrome oxidase subunit II causes defective assembly and myopathy AMERICAN JOURNAL OF HUMAN GENETICS, 65 :1030-1039 DOI Author Url

Hargreaves IP. Simvastatin pre-treatment improves survival and mitochondrial function in a 3-day fluid-resuscitated rat model of sepsis. Clinical science (London, England : 1979), DOI Author Url

Hargreaves IP. Secondary coenzyme Q10 deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders. Mitochondrion, DOI Author Url

Stepien KM, Roncaroli F, Turton N, Hendriksz CJ, Roberts M, Heaton R, Hargreaves I. Mechanisms of Mitochondrial Dysfunction in Lysosomal Storage Disorders: A Review Journal of Clinical Medicine, DOI Public Url

Other

Hargreaves A, Maguire A, Mooney C, Hargreaves I, Heaton R, Phillips S, Gill M. 2020. T58. EFFECTIVENESS OF ORALLY ADMINISTERED CO-ENZYME Q10 FOR SCHIZOPHRENIA: COGNITIVE, FUNCTIONAL AND BIOCHEMICAL OUTCOMES FROM A DOUBLE BLIND, RANDOMISED, PLACEBO CONTROLLED TRIAL Schizophrenia Bulletin, 46 :S253-S253 DOI Publisher Url

Chapters

Hargreaves I. 2020. Coenzyme Q10 Assessment and the Establishment of a Neuronal Cell Model of CoQ10 Deficiency guest P. Clinical and Preclinical Models for Maximizing Healthspan :277-287 Springer DOI Author Url Publisher Url Public Url

Hargreaves IP, Mantle D. 2019. Coenzyme Q10 Supplementation in Fibrosis and Aging Guest PC. REVIEWS ON BIOMARKER STUDIES IN AGING AND ANTI-AGING RESEARCH 1178 :103-112 SPRINGER INTERNATIONAL PUBLISHING AG 978-3-030-25649-4 DOI Author Url

Neergheen V, Dyson A, Wainwright L, Hargreaves IP. 2018. Statin and Fibrate-Induced Dichotomy of Mitochondrial Function Mitochondrial Dysfunction Caused by Drugs and Environmental Toxicants 2-2 :457-473 9781119329701 DOI

Neergheen V, Hargreaves IP. 2018. Secondary coenzyme Q10 deficiency: Causes and consequence Coenzyme Q10: Uses, Health Effects and Role in Disease :89-110 9781536132847

Yubero D, Montero R, O’Callaghan M, Pineda M, Meavilla S, Delgadillo V, Sierra C, Altimira L, Navas P, Pope S, Oppenheim M, Neergheen V, Ghosh A, Mills P, Clayton P, Footitt E, Cleary M, Hargreaves I, Jones SA, Heales S, Artuch R. 2016. Coenzyme Q10 and pyridoxal phosphate deficiency is a common feature in mucopolysaccharidosis type III JIMD Reports 25 :1-7 DOI

Book

Hargreaves IP. 2019. Mitochondrial Respiratory Chain Disorders From Clinical Presentation to Diagnosis and Treatment 9781536150537

Hargreaves IP. 2015. Coenzyme Q10: From fact to fiction :1-356 9781634828222

Conference publication

Chambers D, Kumar A, Feng L, Hargreaves I, Lam A, Heales S, Manzur A, Muntoni F, Sewry C, Poulton J, Phadke R. 2019. A novel multiplex chromogenic immunoassay for evaluating mitochondrial respiratory chain complex I and complex IV defects in diagnostic muscle biopsies NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 120th Meeting of the British-Neuropathological-Society (BNS) / Developmental Neuropathology Symposium 45 :27-28 Author Url

Chambers D, Kumar A, Feng L, Hargreaves I, Lam A, Manzur A, Muntoni F, Sewry C, Poulton J, Phadke R. 2018. A novel multiplex chromogenic immunoassay for evaluating mitochondrial respiratory chain complex I and complex IV defects in diagnostic muscle biopsies NEUROMUSCULAR DISORDERS, 23rd International Annual Congress of the World-Muscle-Society (WMS) 28 :S86-S86 DOI Author Url

Lam A, Neergheen V, Chalasani A, Salih K, Pope S, Hargreaves I, Prunty H, Heales S. 2018. Biochemical markers of primary mitochondrial respiratory chain enzyme disorders NEUROMUSCULAR DISORDERS, 11th UK Neuromuscular Translational Research Conference 28 :S31-S31 DOI Author Url

Foti S, Carrington S, Kiely A, Houlden H, Hargreaves I, Holton J. 2018. Cerebral mitochondrial electron transport chain dysfunction in multiple system atrophy NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 119th Meeting of the British-Neuropathological-Society (BNS) / Epilepsy Neuropathology Symposium 44 :15-15 Author Url

Keatley K, Lurshay S, Polyzoidis S, Ashkan K, Fillmore H, Pilkington G, Hargreaves I, Meunier B, McGeehan J, Lloyd R. 2016. SINGLE MITOCHONDRIAL DNA VARIATIONS INFLUENCE MITOCHONDRIAL TARGETED DRUG SENSITIVITY IN IN VITRO MODELS OF GLIOBLASTOMA NEURO-ONCOLOGY, 21st Annual Scientific Meeting and Education Day of the Society-for-Neuro-Oncology 18 :82-82 Author Url

Poole OV, Fernandez-Vizarra E, Turner C, Clarke B, Bugiardini E, Barbosa IA, Deshpande C, Hargreaves IP, Woodward CE, Sweeney MG, Poulton J, Simpson MA, Houlden H, Zeviani M, Hanna MG, Pitceathly RDS. 2016. TRIAP1 MUTATIONS ARE A CAUSE OF REVERSIBLE INFANTILE BULBAR FAILURE WITH SUBSEQUENT PROGRESSIVE ADOLESCENT ONSET MYOPATHY MUSCLE & NERVE, Annual Meeting of the Muscle-Study-Group on Models of Neuromuscular Disease scross the Lifespan 54 :14-14 Author Url

Bugiardini E, Poole O, Manole A, Horga A, Hargreaves I, Holton JL, Woodward CE, Sweeney MG, Pittman A, Taylor J, Smith C, Fratter C, Poulton J, Plant G, Spinazzola A, Holt IJ, Houlden H, Hanna MG, Pitceathly RDS. 2016. RNASEH1 MUTATIONS ARE A RARE CAUSE OF CPEO WITH MULTIPLE MTDNA DELETIONS MUSCLE & NERVE, Annual Meeting of the Muscle-Study-Group on Models of Neuromuscular Disease scross the Lifespan 54 :15-15 Author Url

Smith KJ, Davies A, Desai RA, Sadeghian M, Amatruda M, Tachrount M, Kaynezhad P, Schiza D, Sajic M, Hargreaves I, Xiulin N, Heales S, Kasti M, Diem R, Tachtsidis I, Golay X, Duchen M. 2016. Multiple sclerosis from an energy perspective. MULTIPLE SCLEROSIS JOURNAL, 32nd Congress of the European-Committee-for-Treatment-and-Research-in-Multiple-Sclerosis (ECTRIMS) 22 :37-38 Author Url

Wood-Kaczmar A, Ghosh R, Kriston-Vizi J, Smith EJ, Chadwick W, Hargreaves IP, Heales SJ, Abramov AY, Cole SL, Ketteler R, Bates GP, Andre R, Tabrizi SJ. 2016. ABNORMAL BIOENERGETICS IN INCLUSION-CONTAINING MUTANT HTT EXON 1 PRIMARY HUMAN NEURONS JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 87 :A18-A19 DOI Author Url

Wauters SC, Kiely A, Houlden H, Hargreaves I, Holton JL. 2016. Mitochondrial dysfunction in multiple system atrophy NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 117th Meeting of the British-Neuropathological-Society 42 :37-38 Author Url

Abeti R, Parkinson MH, Hargreaves IP, Pook MA, Abramov AY, Giunti P. 2016. Understanding the Role of Mitochondrial Pathophysiology in Friedreich's Ataxia BIOPHYSICAL JOURNAL, 60th Annual Meeting of the Biophysical-Society 110 :474A-474A DOI Author Url

Keatley K, Polyzoidis S, Ashkan K, Heales SJ, Fillmore HL, Hargreaves IP, McGeehan JE, Lloyd RE, Pilkington GJ. 2015. BIOCHEMICAL ANALYSIS COMBINED WITH 3D STRUCTURAL PROTEIN MODELLING IDENTIFIES THE ROLE OF SPECIFIC MITOCHONDRIAL MUTATIONS IN GBM NEURO-ONCOLOGY, Meeting of the British-Neuro-Oncology-Society (BNOS) 17 :10-11 Author Url

Schottlaender LV, Bettencourt C, Kiely A, Chalasani A, Neergheen V, Holton JL, Hargreaves IP, Houlden H. 2015. Coenzyme Q10 levels are reduced in the cerebellum of multiple system atrophy patients MOVEMENT DISORDERS, 19th International Congress of Parkinson's Disease and Movement Disorders 30 :S328-S329 Author Url

Murray CE, Pressey SN, Heywood WE, Hargreaves IP, Neergheen V, Wauters S, Palkovits M, Gelpi E, Troakes C, Gentleman SM, Mills K, Holton JL, Revesz T, Gandhi S. 2015. Mitochondrial dysfunction in Parkinson's disease: Is it the earliest feature? NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 116th Meeting of the British-Neuropathological-Society 41 :11-11 Author Url

Cortese A, Ellis M, Chambers D, Rahman S, Hargreaves I, Fratter C, Sewry CA, Muntoni F, Poulton J, Phadke R. 2014. The confounding effect of age in the use of subsarcolemmal mitochondrial aggregates (SSMA) as a diagnostic muscle biopsy marker in paediatric mitochondrial disease NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 115th Meeting of the British-Neuropathological-Society, Institute-of-Child-Health 40 :16-16 Author Url

Dunn L, Allen G, Ling H, Li A, Duberly K, Hargreaves I, Lees A, Holton JL, Heales S, Bandopadhyay R. 2014. Perturbed glucose metabolism in Parkinson's disease NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 115th Meeting of the British-Neuropathological-Society, Institute-of-Child-Health 40 :13-14 Author Url

Brealey D, Hargreaves I, Land J, Singer M, Mc Auley D. 2012. PLASMA UBIQUINONE LEVELS ARE UNAFFECETED BY SIMVASTATIN IN ACUTE LUNG INJURY INTENSIVE CARE MEDICINE, 38 :S64-S64 Author Url

Hargreaves IP, Chasalani A, Heales SJ, Land JM. 2012. COMPLEX V DEFICIENCY AND MIS-ASSEMBLY: A CASE FOR INCREASED INVESTIGATION UTILISING BLUE NATIVE GEL ANALYSIS JOURNAL OF INHERITED METABOLIC DISEASE, 35 :S115-S115 Author Url

Aylett S-B, Varadkar S, Fassone E, Pope S, Neergheen V, Hargreaves IP, Land JM, Heales SJ, Rahman S. 2012. CEREBRAL FOLATE DEFICIENCY ASSOCIATED WITH A NOVEL HOMOZYGOUS NONSENSE MUTATION IN FOLR1 JOURNAL OF INHERITED METABOLIC DISEASE, 35 :S138-S138 Author Url

Fassone E, Taanman JW, Sweeney MG, Woodward C, Hargreaves IP, Hanna MG, Taylor RW, Duncan AJ, Rahman S. 2012. WHOLE EXOME SEQUENCING REVEALS THAT SUBUNIT MUTATIONS ARE PREVALENT IN COMPLEX I DEFICIENT LEIGH SYNDROME JOURNAL OF INHERITED METABOLIC DISEASE, 35 :S13-S13 Author Url

Pitceathly RDS, Tomlinson SE, Hargreaves I, Bhardwaj N, Holton JL, Evans J, Smith C, Fratter C, Woodward C, Sweeney MG, Hanna MG, Rahman S. 2012. NOVEL DOMINANT POLG MUTATIONS CAUSE DISTAL MYOPATHY JOURNAL OF INHERITED METABOLIC DISEASE, 35 :S119-S119 Author Url

Hargreaves I, Land J. 2012. Multiple mitochondrial electron transport chain enzyme deficiencies associated with a decrease in skeletal muscle coenzyme Q10 status MITOCHONDRION, 12 :590-591 DOI Author Url

Hargreaves IP, Heales SJR, Duncan AJ, Land JM. 2012. MITOCHONDRIAL ELECTRON TRANSPORT CHAIN DYSFUNCTION ASSOCIATED WITH STATIN THERAPY JOURNAL OF INHERITED METABOLIC DISEASE, 35 :S121-S121 Author Url

Christodoulou J, Gold WA, Williamson SL, Kaur S, Gibson JH, Pelka GJ, Hargreaves IP, Land JM, Tam PPL, Christodoulou J. 2012. Mitochondrial defects in Rett syndrome MITOCHONDRION, 12 :576-577 DOI Author Url

Duberley KEC, Hargreaves IP, Chaiwatanasirikul K, Heales SJ, Rahman S, Mills K, Eaton S. 2012. DEVELOPMENT OF A MASS SPECTROMETRY METHOD (MS/MS) FOR QUANTIFICATION OF COENZYME Q10 IN CSF JOURNAL OF INHERITED METABOLIC DISEASE, 35 :S124-S124 Author Url

Munteanu I, Zhou H, Hargreaves I, Yasukawa T, Duchen MR, Muntoni F. 2012. Investigating mitochondria in cell culture models of core myopathies NEUROMUSCULAR DISORDERS, United Kingdom Neuromuscular Translational Research Conference 22 :S30-S30 Author Url

Foley AR, Broomfield AA, Pandraud A, Johnson JO, Singleton AB, Hargreaves IP, Land JM, Grunewald S, Rahman S, Clayton P, Houlden H, Reilly MM, Muntoni F. 2012. High-dose riboflavin therapy in Brown-Vialetto-Van Laere syndrome: clinical and biochemical improvement NEUROMUSCULAR DISORDERS, United Kingdom Neuromuscular Translational Research Conference 22 :S4-S4 Author Url

Pitceathly RDS, Murphy SM, Cottenie E, Chalasani A, Sweeney MG, Woodward C, Mudanohwo EE, Hargreaves I, Heales S, Holton JL, Houlden H, Lunn MP, Rahman S, Reilly MM, Hanna MG. 2012. Genetic dysfunction of MT-ATP6 can cause axonal Charcot-Marie-Tooth disease NEUROMUSCULAR DISORDERS, United Kingdom Neuromuscular Translational Research Conference 22 :S20-S20 Author Url

Pandraud A, Johnson JO, Singleton AB, Clayton P, Land J, Hargreaves I, Foley AR, Muntoni F, Reilly MM, Houlden H. 2012. Genetic and functional investigation of Brown-Vialetto-Van Laere syndrome and related neuropathies NEUROMUSCULAR DISORDERS, United Kingdom Neuromuscular Translational Research Conference 22 :S19-S20 Author Url

Hargreaves IP, Land JM. 2011. MULTIPLE MITOCHONDRIAL ELECTRON TRANSPORT CHAIN ENZYME DEFICIENCIES ASSOCIATED WITH A DECREASE IN SKELETAL MUSCLE COENZYME Q10 STATUS JOURNAL OF INHERITED METABOLIC DISEASE, 34 :S164-S164 Author Url

Duberley KEC, Abramov A, Heales SJR, Rahman S, Hargreaves IP. 2011. ESTABLISHMENT OF A NEURONAL CELL MODEL OF COENZYME Q10 DEFICIENCY: IMPLICATIONS FOR THE PATHOGENESIS OF DEFECTS IN COENZYME Q10 BIOSYNTHESIS JOURNAL OF INHERITED METABOLIC DISEASE, 34 :S164-S164 Author Url

Rajakulendran S, Pitceathly RDS, Warren J, Woodward C, Sweeney MG, Hargreaves I, Fratter C, Heales S, Taylor R, Holton JL, Rahman S, Hanna MG. 2010. MULTIPLE MITOCHONDRIAL DNA DELETIONS, CYCLOOXYGENASE-NEGATIVE FIBRES AND SLOWLY PROGRESSIVE COGNITIVE DECLINE WITH PSYCHIATRIC FEATURES JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, Annual Meeting of the Association-of-British-Neurologists 81 DOI Author Url

Pitceathly RDS, Holton JL, Hargreaves I, Heales S, Woodward C, Sweeney MG, Davis MB, Evans J, Smith C, Fratter C, Rahman S, Hanna MG. 2010. Novel POLG mutation causing distal myopathy and cachexia JOURNAL OF MEDICAL GENETICS, British Human Genetics Conference 47 :S54-S54 Author Url

Duberley KEC, Heales SJR, Rahman S, Allen G, Hargreaves IP. 2010. ESTABLISHMENT OF A NEURONAL CELL MODEL OF COENZYME Q10 DEFICIENCY: IMPLICATIONS FOR PATHOGENESIS AND TREATMENT OF DISORDERS OF COENZYME Q10 BIOSYNTHESIS JOURNAL OF INHERITED METABOLIC DISEASE, 33 :S80-S80 Author Url

Hargreaves IP, Lynes GW, Land JM. 2010. ASSESSMENT OF CARNITINE PALMITOYLTRANSFERASE II ACTIVITY IN SMALL SKELETAL MUSCLE BIOPSIES JOURNAL OF INHERITED METABOLIC DISEASE, 33 :S59-S59 Author Url

Rahman S, Duncan AJ, Bitner-Glindziez M, Meunier B, Costello H, Hargreaves IP, Lopez LC, Hirano M, Quinzii CM, Sadowski MI, Singleton A, Clayton PT. 2009. A NEW GENETIC CAUSE OF PRIMARY COENZYME Q10 DEFICIENCY MOLECULAR GENETICS AND METABOLISM, 11th International Conference of Inborn Errors of Metabolism 98 :5-5 Author Url

Briddon T, Hargreaves IP. 2009. Oxidative interactions of aminothiols in buffer versus physiological fluid AMINO ACIDS, 37 :34-34 Author Url

Wood-Kaczmar A, Gandhi S, Yao Z, Abramov AY, Miljan EA, Keen G, Stanyer L, Hargreaves I, Klupsch K, Downward J, Mansfield L, Jat P, Taylor J, Heales S, Duchen MR, Latchman D, Tabrizi SJ, Wood NW. 2009. PINK1 is necessary for long term survival and mitochondrial function in primary human dopaminergic neurons JOURNAL OF NEUROCHEMISTRY, 3rd International-Society-of-Neurochemistry-Special-Neurochemistry Conference/8th International Meeting on Brain Energy Metabolism 109 :301-302 Author Url

Yao Z, Klupsh K, Hargreaves I, Downward J, Heales S, Revesz T, Holton J, Wood NW. 2009. Characterisation of PINK1 knockout mice JOURNAL OF NEUROCHEMISTRY, 3rd International-Society-of-Neurochemistry-Special-Neurochemistry Conference/8th International Meeting on Brain Energy Metabolism 109 :300-300 Author Url

Lynes G, Hargreaves IP, Land JM. 2008. The diagnostic of skeletal muscle acylcarnitine profiles in identifying defects of fatty acid beta-oxidation JOURNAL OF INHERITED METABOLIC DISEASE, 31 :38-38 Author Url

Pope SAS, Chalasani A, Lyrics GW, Hargreaves IP, Oppenheim MLS, Hanna MG, Land JM, Heales SJR. 2007. Mitochondrial dysfunction in association with cardiolipin deficiency JOURNAL OF INHERITED METABOLIC DISEASE, 30 :70-70 Author Url

Land JM, Hargreaves IP, Oppenheim M, Pope S, Heales SJR. 2007. Inherited mitochondrial disease, pathogenesis and therapeutic approaches JOURNAL OF NEUROCHEMISTRY, 17th European-Society-for-Neurochemistry Meeting/3rd Conference on Advances in Molecular Mechanisms of Neurological Disorder 101 :14-14 Author Url

Gandhi S, Muqit MMK, Abou-Sleiman PM, Stanyer L, Hargreaves I, Heales S, Ganguly M, Latchman DS, Holton J, Wood NW, Revesz T. 2006. PINK1: a novel mitochondrial protein in Parkinson's disease NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 107th Meeting of the British-Neuropathological-Society 32 :221-222 Author Url

Hargreaves IP, Duncan A, Mills K, Eaton S, Land JM, Heales SJR. 2005. Blood mononuclear cell, but not plasma coenzyme Q(10) concentration correlates with skeletal muscle levels: implications for diagnosis and treatment JOURNAL OF THE NEUROLOGICAL SCIENCES, 18th World Congress of Neurology 238 :S170-S171 Author Url

Liolitsa D, McKenzie M, Hargreaves I, Heales S, Land J, Sisodiya SM, Wood NW, Duchen MR, Hanna MG. 2005. MELAS, a young-onset stroke disorder associated with a homoplasmic ND5 mitochondrial DNA mutation EUROPEAN JOURNAL OF NEUROLOGY, 12 :158-159 Author Url

Hargreaves IP, Duncan AJ, Wu L, Agrawal A, Land JM, Heales SJR. 2005. INHIBITION OF MITOCHONDRIAL COMPLEX IV LEADS TO SECONDARY LOSS COMPLEX II-III ACTIVITY: IMPLICATIONS FOR THE PATHOGENESIS AND TREATMENT OF MITOCHONDRIAL CYTOPATHIES JOURNAL OF INHERITED METABOLIC DISEASE, 28 :137-137 Author Url

Duncan AJ, Hargreaves IP, Clark JB, Heales SJR. 2003. Nitric oxide increases ubiquinone synthesis in the C6 glioma cell line JOURNAL OF NEUROCHEMISTRY, Joint Meeting of the International-Society-for-Neurochemistry/Asian-Pacific-Society-for-Neurochemistry 87 :155-155 Author Url

Land JM, Hargreaves IP, Brearley D, Heales SJ, Singer M. 2002. Mitochondrial dysfunction in sepsis JOURNAL OF THE NEUROLOGICAL SCIENCES, 10th International Congress on Neuromuscular Diseases 199 :S51-S52 Author Url

Edwards MJ, Hargreaves IP, Heales SJ, Jones SJ, Ramachandran V, Bhatia KP. 2002. The treatment of Unverricht-Lundborg disease with N-acetylcysteine MOVEMENT DISORDERS, 7th International Congress of Parkinsons Disease and Movement Disorders 17 :S334-S334 Author Url

Brealey DA, Brand M, Hargreaves I, Heales S, Land J, Smolenski R, Singer M. 2001. Mitochondrial inhibition is associated with the severity of human septic shock INTENSIVE CARE MEDICINE, 27 :S137-S137 Author Url

Brealey D, Brand M, Hargreaves I, Heales S, Land J, Davies N, Cooper C, Singer M. 2001. Mitochondrial inhibition is associated with the severity of human septic shock BRITISH JOURNAL OF ANAESTHESIA, 87 :340P-341P Author Url

Hargreaves IP, Heales SJR, Olpin SE, Morgan-Hughes JA, Land JM. 2000. The diagnosis of carnitine palmitoyltransferase II deficiency is now possible in small skeletal muscle biopsies JOURNAL OF INHERITED METABOLIC DISEASE, 23 :352-354 DOI Author Url

Heales SJR, Barker JE, Stewart VC, Brand MP, Hargreaves IP, Foppa P, Land JM, Clark JP, Bolanos JP. 1997. Nitric oxide, energy metabolism and neurological disease BIOCHEMICAL SOCIETY TRANSACTIONS, Symposium on Xanthine Oxidase - Enzymology and Pathophysiology, at the 661st Meeting of the Biochemical-Society 25 :939-943 DOI Author Url