Our Publications

Harvey C, Weinreich M, Lee JAK, Shaw AC, Ferraiuolo L, Mortiboys H, Zhang S, Hop PJ, Zwamborn RAJ, van Eijk K , Julian TH et al (2024) Rare and common genetic determinants of mitochondrial function determine severity but not risk of amyotrophic lateral sclerosis. Heliyon, 10(3), e24975-e24975.   

• Bell SM, Wareing H, Hamshaw A, De S, New E, Shaw P, De Marco M, Venneri A, Blackburn DJ, Ferraiuolo L & Mortiboys H (2023) Deficits in mitochondrial function and glucose metabolism seen in sporadic and familial Alzheimer’s disease derived Astrocytes are ameliorated by increasing hexokinase 1. Alzheimer's & Dementia, 19(S13).  

• Payne T, Burgess T, Bradley S, Roscoe S, Sassani M, Dunning MJ, Hernandez D, Scholz S, McNeill A, Taylor R , Su L et al (2023) Multimodal assessment of mitochondrial function in Parkinson's disease. Brain, awad364.  

• Capriglia F, Burgess T, Bandmann O & Mortiboys H (2023) Clinical Trial Highlights: Modulators of Mitochondrial Function. Journal of Parkinson's Disease, 13(6), 851-864.  

• Gonzalez-Robles C, Weil RS, van Wamelen D, Bartlett M, Burnell M, Clarke CS, Hu MT, Huxford B, Jha A, Lambert C , Lawton M et al (2023) Outcome Measures for Disease-Modifying Trials in Parkinson’s Disease: Consensus Paper by the EJS ACT-PD Multi-Arm Multi-Stage Trial Initiative. Journal of Parkinson's Disease, 13(6), 1011-1033.  

• Keatinge M, Gegg ME, Watson L, Mortiboys H, Li N, Dunning M, Ailani D, Bui H, van Rens A, Lefeber DJ , Schapira AHV et al (2023) Unexpected phenotypic and molecular changes of combined glucocerebrosidase and acid sphingomyelinase deficiency. Disease Models & Mechanisms, 16(6).  

• Elcocks H, Brazel AJ, McCarron KR, Kaulich M, Husnjak K, Mortiboys H, Clague MJ & Urbé S (2023) FBXL4 ubiquitin ligase deficiency promotes mitophagy by elevating NIX levels. The EMBO Journal.  

• Morsli S, Henriques CM, Ellis PS, Mortiboys H, Baxendale S, Loynes CA, Renshaw SA & Bellantuono I (2023) A p21‐GFP zebrafish model of senescence for rapid testing of senolytics in vivo. Aging Cell.  

• Payne T, Burgess T, Sassani M, Bradley S, Roscoe S, Reed E, Wilkinson ID, Mortiboys H, Jenkins TM & Bandmann O (2022) Using 31-phosphorus magnetic resonance spectroscopy (31P-MRS) to identify Parkinson’s Disease subgroups with bioenergetic dysfunction. Journal of Neurology, Neurosurgery & Psychiatry, 93(9), e2.2-e2.2.  

• Rusilowicz-Jones EV, Barone FG, Martins Lopes F, Stephen E, Mortiboys H, Urbé S & Clague MJ (2022) Benchmarking a highly selective USP30 inhibitor for enhancement of mitophagy and pexophagy. Life Science Alliance, 5(2).  

• Sassani M, Alix J, Mcdermott C, Baster K, Hoggard N, Wild J, Mortiboys H, Shaw P, Wilkinson I & Jenkins T (2021) Brain energy metabolism in ALS: A phosphorus-31 magnetic resonance spectroscopy study. Journal of the Neurological Sciences, 429, 119412.  

• Frison M, Faccenda D, Abeti R, Rigon M, Strobbe D, England-Rendon BS, Cash D, Barnes K, Sadeghian M, Sajic M , Wells LA et al (2021) The translocator protein (TSPO) is prodromal to mitophagy loss in neurotoxicity. Molecular Psychiatry. View this article in WRRO  

• Leah T, Vazquez-Villaseñor I, Ferraiuolo L, Wharton S & Mortiboys H (2021) A Parkinson’s disease-relevant mitochondrial and neuronal morphology high-throughput screening assay in LUHMES cells. Bio-protocol, 11(1).  

• Gatto N, Dos Santos Souza C, Shaw AC, Bell SM, Myszczynska MA, Powers S, Meyer K, Castelli LM, Karyka E, Mortiboys H , Azzouz M et al (2020) Directly converted astrocytes retain the ageing features of the donor fibroblasts and elucidate the astrocytic contribution to human CNS health and disease. Aging Cell. View this article in WRRO  

• Bell SM, De Marco M, Barnes K, Shaw P, Ferraiuolo L, Blackburn DJ, Mortiboys H & Venneri A (2020) Do deficits in mitochondrial spare respiratory capacity contribute to neuropsychological changes seen in Alzheimer’s disease?. Alzheimer's & Dementia, 16(S6).  

• Bell SM, Burgess T, Lee J, Blackburn DJ, Allen SP & Mortiboys H (2020) Peripheral glycolysis in neurodegenerative diseases. International Journal of Molecular Sciences, 21(23).  

• Schwartzentruber A, Boschian C, Lopes FM, Myszczynska MA, New EJ, Beyrath J, Smeitink J, Ferraiuolo L & Mortiboys H (2020) Oxidative switch drives mitophagy defects in dopaminergic parkin mutant patient neurons. Scientific Reports, 10(1).  

• Rusilowicz-Jones EV, Jardine J, Kallinos A, Pinto-Fernandez A, Guenther F, Giurrandino M, Barone FG, McCarron K, Burke CJ, Murad A , Martinez A et al (2020) USP30 sets a trigger threshold for PINK1–PARKIN amplification of mitochondrial ubiquitylation. Life Science Alliance, 3(8). View this article in WRRO  

• Bell SM, De Marco M, Barnes K, Shaw PJ, Ferraiuolo L, Blackburn DJ, Mortiboys H & Venneri A (2020) Deficits in mitochondrial spare respiratory capacity contribute to the neuropsychological changes of alzheimer’s disease. Journal of Personalized Medicine, 10(2). View this article in WRRO  

• Bell S, De Marco M, Barnes K, Shaw P, Ferraiuolo L, Blackburn D, Mortiboys H & Venneri A (2020) Do deficits in Mitochondrial Spare Respiratory Capacity contribute to Neuropsychological changes seen in Alzheimer’s disease? (798). Neurology, 94(15_supplement).  

• Carling PJ, Mortiboys H, Green C, Mihaylov S, Sandor C, Schwartzentruber A, Taylor R, Wei W, Hastings C, Wong S , Lo C et al (2020) Deep phenotyping of peripheral tissue facilitates mechanistic disease stratification in sporadic Parkinson’s disease. Progress in Neurobiology, 187.  

• Allen SP, Hall B, Woof R, Francis L, Gatto N, Shaw AC, Myszczynska M, Hemingway J, Coldicott I, Willcock A , Job L et al (2019) C9orf72 expansion within astrocytes reduces metabolic flexibility in amyotrophic lateral sclerosis. Brain, 1-20. View this article in WRRO  

• López KLR, Simpson JE, Watson LC, Mortiboys H, Hautbergue GM, Bandmann O & Highley JR (2019) TIGAR inclusion pathology is specific for Lewy body diseases. Brain Research, 1706, 218-223. View this article in WRRO  

• Bell SM, Barnes K, Clemmens H, Al-Rafiah AR, Al-ofi EA, Leech V, Bandmann O, Shaw PJ, Blackburn DJ, Ferraiuolo L & Mortiboys H (2018) Ursodeoxycholic Acid Improves Mitochondrial Function and Redistributes Drp1 in Fibroblasts from Patients with either Sporadic or Familial Alzheimer's Disease. Journal of Molecular Biology, 430(21), 3942-3953. View this article in WRRO  

• Ratcliffe LE, Vázquez Villaseñor I, Jennings L, Heath PR, Mortiboys H, Schwartzentruber A, Karyka E, Simpson JE, Ince PG, Garwood CJ & Wharton SB (2018) Loss of IGF1R in human astrocytes alters complex I activity and support for neurons.. Neuroscience, 390, 46-59. View this article in WRRO  

• Macdonald R, Barnes K, Hastings C & Mortiboys H (2018) Mitochondrial abnormalities in Parkinson's disease and Alzheimer's disease: can mitochondria be targeted therapeutically?. Biochemical Society Transactions, 46(4), 891-909. View this article in WRRO  

• Mortiboys H, Macdonald R, Payne T, Sassani M, Jenkins T & Bandmann O (2018) Translational approaches to restoring mitochondrial function in Parkinson's disease. FEBS Letters, 592(5), 776-792. View this article in WRRO  

• Carling P, Mortiboys H, Mihaylov S, Clemmens H, Schwartzenhuber A, Wade-Martins R, Talbot K, Hu M & Bandmann O (2017) PO076 Disease stratification in sporadic parkinson’s disease. Journal of Neurology, Neurosurgery & Psychiatry, 88(Suppl 1), A31.4-A32.  

• Bell SM, Clemmens H, Blackburn DJ, Bandmann O, Ferraiuolo L & Mortiboys H (2017) [P4-034]: MITOCHONDRIAL ABNORMALITIES ARE FOUND IN FIBROBLASTS FROM SPORADIC ALZHEIMER's DISEASE PATIENTS: RECOVERY WITH URSODOXYCHOLIC ACID TREATMENT. Alzheimer's & Dementia, 13(7S_Part_26), P1269-P1269.  

• Dombi E, Mortiboys H & Poulton J (2017) Modulating mitophagy in mitochondrial disease.. Curr Med Chem. View this article in WRRO  

• Soman S, Keatinge M, Moein M, DaCosta M, Mortiboys H, Skupin A, Sugunan S, Bazala M, Kuznicki J & Bandmann O (2017) Inhibition of the mitochondrial calcium uniporter (MCU) rescues dopaminergic neurons in pink1-/- zebrafish. European Journal of Neuroscience, 45(4), 528-535. View this article in WRRO  

• Liao C, Ashley N, Diot A, Morten K, Phadwal K, Williams A, Fearnley I, Rosser L, Lowndes J, Fratter C , Ferguson DJ et al (2017) Dysregulated mitophagy and mitochondrial organization in optic atrophy due to OPA1 mutations. Neurology, 88(2), 131-142. View this article in WRRO  

• Mortiboys H (2016) Screening for chemical modulators for LRRK2.. Biochemical Society Transactions, 44(6), 1617-1623. View this article in WRRO  

• Yealland G, Battaglia G, Bandmann O & Mortiboys H (2016) Rescue of mitochondrial function in parkin-mutant Fibroblasts using drug loaded PMPC-PDPA polymersomes and tubular polymersomes. Neuroscience Letters, 630, 23-29. View this article in WRRO  

• Keatinge M, Bui H, Menke A, Chen Y-C, Sokol AM, Bai Q, Ellett F, Da Costa M, Burke D, Gegg M , Trollope L et al (2015) Glucocerebrosidase 1 deficient Danio rerio mirror key pathological aspects of human Gaucher disease and provide evidence of early microglial activation preceding alpha-synuclein-independent neuronal cell death. Human Molecular Genetics, 24(23), 6640-6652. View this article in WRRO  

• Diot A, Hinks-Roberts A, Lodge T, Liao C, Dombi E, Morten K, Brady S, Fratter C, Carver J, Muir R , Davis R et al (2015) A novel quantitative assay of mitophagy: Combining high content fluorescence microscopy and mitochondrial DNA load to quantify mitophagy and identify novel pharmacological tools against pathogenic heteroplasmic mtDNA. Pharmacological Research, 100, 24-35.  

• Mortiboys H, Furmston R, Bronstad G, Aasly J, Elliott C & Bandmann O (2015) UDCA exerts beneficial effect on mitochondrial dysfunction in LRRK2G2019S carriers and in vivo. Neurology, 85(10), 846-852.  

• Pogson JH, Ivatt RM, Sanchez-Martinez A, Tufi R, Wilson E, Mortiboys H & Whitworth AJ (2014) The Complex I Subunit NDUFA10 Selectively Rescues Drosophila pink1 Mutants through a Mechanism Independent of Mitophagy. PLoS Genetics, 10(11). View this article in WRRO  

• Hickey FB, Corcoran JB, Griffin B, Bhreathnach U, Mortiboys H, Reid HM, Andrews D, Byrne S, Furlong F, Martin F , Godson C et al (2014) IHG-1 increases mitochondrial fusion and bioenergetic function.. Diabetes, 63(12), 4314-4325.  

• Bandmann O, Keatinge M, DaCosta M, Mortiboys H & Flinn L (2014) TIGAR Inhibition Rescues Dopaminergic Neurons in Common Forms of Early Onset Parkinson's' Disease (S7.002). Neurology, 82(10_supplement).  

• Bandmann O, Keatinge M, DaCosta M, Mortiboys H & Flinn L (2014) TIGAR Inhibition Rescues Dopaminergic Neurons in Common Forms of Early Onset Parkinson's' Disease (I5-2.003). Neurology, 82(10_supplement).  

• Hinks-Roberts A, Dombi E, Diot A, Liao C, Morten K, Carver J, Lodge T, Mortiboys H & Poulton J (2014) P33 Do modulators of mitophagy select pathogenic mtDNA mutations?. Neuromuscular Disorders, 24, S15-S15.  

• Allen SP, Rajan S, Duffy L, Mortiboys H, Higginbottom A, Grierson AJ & Shaw PJ (2014) Superoxide dismutase 1 mutation in a cellular model of amyotrophic lateral sclerosis shifts energy generation from oxidative phosphorylation to glycolysis.. Neurobiol Aging, 35(6), 1499-1509.  

• Mortiboys H, Aasly J & Bandmann O (2013) Ursocholanic acid rescues mitochondrial function in common forms of familial Parkinson's disease.. Brain, 136(Pt 10), 3038-3050.  

• Flinn LJ, Keatinge M, Bretaud S, Mortiboys H, Matsui H, De Felice E, Woodroof HI, Brown L, McTighe A, Soellner R , Allen CE et al (2013) TigarB causes mitochondrial dysfunction and neuronal loss in PINK1 deficiency.. Ann Neurol, 74(6), 837-847. View this article in WRRO  

• Richardson K, Allen SP, Mortiboys HJ, Grierson AJ, Wharton SB, Ince PG, Shaw PJ & Heath PR (2013) The Effect of SOD1 Mutation on Cellular Bioenergetic Profile and Viability in Response to Oxidative Stress and Influence of Mutation-Type.. Plos One, 6(8), e68256-e68256. View this article in WRRO  

• Mortiboys H, Johansen KK, Aasly JO & Bandmann O (2010) Mitochondrial impairment in patients with Parkinson disease with the G2019S mutation in LRRK2.. Neurology, 75(22), 2017-2020.  

• Bandmann O, Flinn L & Mortiboys H (2010) POMD08 Zebrafish models for early onset Parkinson's disease.. J Neurol Neurosurg Psychiatry, 81(11), e59.  

• Bandmann O, Flinn L & Mortiboys H (2010) A PINK1 Mutant Zebrafish Model of Early Onset Parkinson's Disease. MOVEMENT DISORD, 25, S631-S631.  

• Cox LE, Ferraiuolo L, Goodall EF, Heath PR, Higginbottom A, Mortiboys H, Hollinger HC, Hartley JA, Brockington A, Burness CE , Morrison KE et al (2010) Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS).. PLoS One, 5(3), e9872. View this article in WRRO  

• Tain LS, Mortiboys H, Tao RN, Ziviani E, Bandmann O & Whitworth AJ (2009) Rapamycin activation of 4E-BP prevents parkinsonian dopaminergic neuron loss.. Nature Neuroscience, 12(9), 1129-1135. View this article in WRRO  

• Flinn L, Mortiboys H, Volkmann K, Köster RW, Ingham PW & Bandmann O (2009) Complex I deficiency and dopaminergic neuronal cell loss in parkin-deficient zebrafish (Danio rerio).. Brain, 132(Pt 6), 1613-1623.  

• Mortiboys H, Thomas KJ, Koopman WJH, Klaffke S, Abou-Sleiman P, Cookson MR & Bandmann O (2009) 7. Mitochondrial function and morphology are impaired in parkin mutant fibroblasts. Mitochondrion, 9(1), 63-63.  

• Mortiboys H, Thomas KJ, Koopman WJH, Klaffke S, Abou-Sleiman P, Olpin S, Wood NW, Willems PHGM, Smeitink JAM, Cookson MR & Bandmann O (2008) Mitochondrial function and morphology are impaired in parkin-mutant fibroblasts.. Ann Neurol, 64(5), 555-565.  

• Osborne NN, Li G-Y, Ji D, Mortiboys HJ & Jackson S (2008) Light affects mitochondria to cause apoptosis to cultured cells: possible relevance to ganglion cell death in certain optic neuropathies.. J Neurochem, 105(5), 2013-2028.  

• Mortiboys HJ, Schaefer J, Reichmann H & Jackson S (2007) Mitochondrial dysfunction in Parkinson's disease--revisited.. Neurol Neurochir Pol, 41(2), 150-159.  

• Schaefer J, Mortiboys H, Navas P, Weinhold M, Reichmann H & Jackson S (2006) Primärer Coenzym Q10-Mangel bei einem Erwachsenen. Aktuelle Neurologie, 33(S 1).  

• Mihaylov SR, Castelli LM, Lin Y-H, Gül A, Soni N, Hastings C, Flynn HR, Păun O, Dickman MJ, Snijders AP , Goldstone R et al () The master energy homeostasis regulator PGC-1α exhibits an mRNA nuclear export function. Nature Communications, 14(1).  

• Bellantuono I, Mortiboys H, Rocha E, Chamoli M, Chinta SJ, Andersen JK, Wallis R, Bezard E, Goldberg M, Greenamyre T , Hirst W et al () Aging, Parkinson’s Disease, and Models: What Are the Challenges?. Aging Biology, 1(1), 20230010-20230010.  

• Foltynie T, Gandhi S, Gonzalez-Robles C, Zeissler M-L, Mills G, Barker R, Carpenter J, Schrag A, Schapira A, Bandmann O , Mullin S et al () Towards a multi-arm multi-stage platform trial of disease modifying approaches in Parkinson’s disease. Brain.  

• Luxenburger A, Clemmens H, Hastings C, Harris LD, Ure EM, Cameron SA, Aasly J, Bandmann O, Weymouth-Wilson A, Furneaux RH & Mortiboys H () 3α,7-Dihydroxy-14(13→12)abeo-5β,12α(H),13β(H)-cholan-24-oic Acids Display Neuroprotective Properties in Common Forms of Parkinson’s Disease. Biomolecules, 13(1), 76-76.  

• Stephen E & Mortiboys H () Direct Reprogramming Retains Aging Signatures That Are Critical to Reveal Parkinson's Disease-Associated Autophagy Phenotypes. Cellular Reprogramming.  

• Vazquez‐Villasenor I, Garwood CJ, Simpson JE, Heath PR, Mortiboys H & Wharton SB () Persistent DNA damage alters the neuronal transcriptome suggesting cell cycle dysregulation and altered mitochondrial function. European Journal of Neuroscience.  

• Schaefer J, Mortiboys H, Reichmann H & Jackson S () Defekte der mitochondrialen Atmungskette bei idiopathischem Parkinson-Syndrom. Aktuelle Neurologie, 32(S 4).  

• Mortiboys HJ, Cox A, Brock IW & Bandmann O () The common PARK8 mutation LRRK2 G2019S is not a risk factor for breast cancer in the absence of Parkinson's disease.. Journal of Neurology.  

• Sassani M, Alix J, McDermott C, Baster K, Hoggard N, Wild J, Mortiboys H, Shaw P, Wilkinson I & Jenkins T () Magnetic resonance spectroscopy reveals mitochondrial dysfunction in amyotrophic lateral sclerosis. Brain.  

• Bell SM, Barnes K, De Marco M, Shaw PJ, Ferraiuolo L, Blackburn DJ, Venneri A & Mortiboys H () Mitochondrial Dysfunction in Alzheimer’s Disease: A Biomarker of the Future?. Biomedicines, 9(1), 63-63.  

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This section will be updated soon

This section will be updated soon