Bandmann, Oliver - University of Sheffield - Department of Neuroscience

个人简介

2002-present: University of Sheffield/Royal Hallamshire Hospital, Sheffield, UK 1997-2002: Specialist Training in Neurology, Marburg, Germany; 1993-1997: Research Fellow, Institute of Neurology. Queen Square London. PhD on "Genetic Aspects of Parkinsonian Disorders" (Supervisors: Prof A.E. Harding, Prof C.D. Marsden, Prof N.W. Wood) 1992-1993: House officer, Dept Neurology, Klinikum Grosshadern, Munich (Head: Prof T. Brandt) 1991: Qualification at Ludwig Maximilian´s University Munich, Germany

研究领域

The main areas of research within my group are as follows: 1. Mitochondrial dysfunction and compound screen with identification of neuroprotective compounds as candidates for disease-modifying treatment in Parkinson’s Disease: We were the first group worldwide to undertake detailed assessment of mitochondrial function and morphology in both parkin-mutant Parkinson’s Disease and LRRK2-G2019S mutant Parkinson’s Disease. We subsequently undertook the first compound screen in Parkinson’s Disease mutant patient tissue. 2000 drugs were assessed for their rescue effect on mitochondrial function. A clear mode of action (MOA) was identified for a group of compounds which includes the FDA-licensed drug ursodeoxycholic acid (UDCA). For interested members of the public: Please have a look at the official University of Sheffield press release for more information on this project. You may also be interested in listening to a BBC interview with Prof Bandmann. OB Image Fig1. The image shows a fibroblast from a patient with Parkinson’s Disease due to mutations in the parkin gene. The fibroblast has been stained to show the mitochondria in the cell. We see increased branching and interconnectedness of the mitochondrial network in the fibroblasts from patients with parkin mutations compared to controls; this change in morphology of the mitochondria correlates with changes in function. 2. Zebrafish as a new vertebrate animal model for Parkinson’s Disease: IMAGE2We were the first group worldwide to establish a zebrafish model of familial Parkinson’s Disease at the MRC Centre for Developmental and Biomedical Genetics (http://cdbg.shef.ac.uk/) . We subsequently demonstrated that Parkin-deficient zebrafish share key features with human parkin-mutant Parkinson’s Disease patients, namely mitochondrial dysfunction and loss of dopaminergic neurons. Most recently, we have identified up-regulation of TIGAR as novel mechanism leading to mitochondrial dysfunction and dopaminergic cell loss in PINK1 deficiency. Inhibition of TIGAR prevents loss of dopaminergic neurons by normalizing mitochondrial function. TIGAR is therefore a promising novel target for disease-modifying therapy in early onset Parkinson’s Disease. For interested members of the public: Please have a look at the official University of Sheffield press release for more information on this project. 3. Mitochondrial biomarkers in Parkinson’s Disease: We are currently undertaking a detailed assessment of mitochondrial function and morphology in patients with sporadic Parkinson’s Disease. This will hopefully allow to eventually identify those patients with Parkinson’s Disease who are most likely to benefit from medication with mitochondrial rescue drugs. 4. Huntington´s Disease (HD) We are recruiting patients for the EURO-HD REGISTRY study at our multidisciplinary Huntington’s Disease clinic. As part of this, we have been frequently recruiting patients for HD drug trials and other HD studies. 5. Wilson Disease (WD) I have previously contributed to the development of the first validated clinical rating scale for the neurological impairment in Wilson Disease. More recently, we have conducted the first study on the genetic prevalence of Wilson Disease in the UK in collaboration with the Welcome Sanger Institute, Cambridge, UK. This study demonstrated a surprisingly high ATP7B carrier frequency which suggests that WD may be considerably more common than previously thought. In collaboration with the Sheffield Diagnostic Genetics Service, we also identified novel genetic mechanisms in WD patients. In close collaboration with the Wilson disease patient self-help group, we are currently establishing a UK-wide, interdisciplinary Wilson disease network. This network will hopefully allow us to develop national standards for the care of Wilson disease patients and facilitate future research projects.

近期论文

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Mortiboys H, Aasly J, Bandmann O. Ursocholanic acid rescues mitochondrial function in common forms of familial Parkinson’s Disease. Brain, 136:3038-50; 2013. Coffey AJ, Durkie M, Hague S, McLay K, Emmerson J, Lo C, Klaffke S, Joyce CJ, Dhawan A, Hadzic N, Mieli-Vergani G, Kirk R, Elizabeth Allen K, Nicholl D, Wong S, Griffiths W, Smithson S, Giffin N, Taha A, Connolly S, Gillett GT, Tanner S, Bonham J, Sharrack B, Palotie A, Rattray M, Dalton A, Bandmann O: A genetic study of Wilson's disease in the United Kingdom. Brain 136:1476-87; 2013. FlinnLJ, Keatinge K, Bretaud S, MortiboysM, Matsui H, De Felice E, Woodroof H, Brown L, McTighe A, Soellner R, Allen CE, Heath PR, Milo M, Muqit MK, Reichert A, Köster R, Ingham PW, Bandmann O. TigarB causes mitochondrial dysfunction and neuronal loss in PINK1 deficiency. Annals of Neurology, [epub ahead of print]; 2013. Cooper-Knock J, Frolov A, Highley R, Charlesworth G, Kirby J, Milano A, Hartley J, Ince PG, McDermott CJ, Lashley T, Revesz T, Shaw PJ, Wood NW, Bandmann O. C9ORF72 expansions, parkinsonism and Parkinson’s Disease – a clinicopathological study. Neurology, 81;808-811; 2013. Mortiboys H, Johansen KK, Aasly JO, Bandmann O: Mitochondrial impairment in patients with Parkinson disease with the G2019S mutation in LRRK2. Neurology 75:2017-20; 2010. Tain LS, Mortiboys H, Tao RN, Ziviani E, Bandmann O, Whitworth AJ: Rapamycin activation of 4E-BP prevents parkinsonian dopaminergic neuron loss. Nature Neuroscience 12:1129-1135; 2009. Mortiboys H, Thomas KJ, Koopman WJH, Klaffke S, Abou-Sleiman P, Olpin S, Wood NW, Willems PHGM, Smeitink JAM, Cookson MR, Bandmann O: Mitochondrial function and morphology are impaired in parkin mutant fibroblasts. Ann Neurol 64:555-565; 2008.