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Dr Jan-Willem Taanman

  • Telephone:

    020 7794 0500

  • Extension:

    #6 292 x35354

  • Fax:

    020 7472 6829

  • Email:

    j.taanman@ucl.ac.uk

  • Address:

    U3/101, U3/102 UCL - Royal Free Campus,
    Rowland Hill Street,
    London,
    NW3 2PF

  • Appointments:

    Reader in Clinical Neurosciences, Clinical Neuroscience, Institute of Neurology

Research Summary

My research focuses mainly on the biogenesis of mitochondria and the role of mitochondria in disease. Historically, I have worked on: (1) structure-function relationships and assembly of the terminal mitochondrial respiratory chain enzyme complex cytochrome-c oxidase, and (2) mitochondrial DNA depletion syndrome. Since 2005, I have also studied mitochondrial aspects in Parkinson’s disease. More recent research projects include: (1) the biogenesis of mitochondrial respiratory chain complex I, (2) the glycolytic switch in the progression of colorectal cancer, (3) the influence of mitochondrial DNA level on cellular energy metabolism, (4) the generation of stem cell models of Parkinson's disease, and (5) the mitochondrial metabolic signature in peripheral tissues of Huntington’s disease patients.

Research Activities

  • A systematic investigation into the pathogenesis and course of Parkinson's syndrome
  • Biogenesis of mitochondrial respiratory chain complex I: relevance to paediatric neurological disease and pathology
  • Disorders of mitochondrial DNA maintenance
  • Dopaminergic neuronal differentiation of human adipose stem cells
  • Inducible pluripotent stem cells from a family with rapid-onset dystonia-parkinsonism
  • Influence of mitochondrial DNA level on energy metabolism: Implications for mitochondrial disease
  • Mitochondrial and metabolic signature(s) in multiple peripheral tissues of Huntington’s patients
  • The glycolytic switch in the progression of colorectal cancer.

Recent Publications

Displaying 50 most recent publications. For the full list please visit UCL Discovery

  1. Wray S,Self M,NINDS Parkinson's Disease iPSC Consortium ,NINDS Huntington's Disease iPSC Consortium ,NINDS ALS iPSC Consortium ,Lewis PA,Taanman JW,Ryan NS,Mahoney CJ,Liang Y,Devine MJ,Sheerin UM,Houlden H,Morris HR,Healy D,Marti-Masso JF,Preza E,Barker S,Sutherland M,Corriveau RA,D'Andrea M,Schapira AH,Uitti RJ,Guttman M,Opala G,Jasinska-Myga B,Puschmann A,Nilsson C,Espay AJ,Slawek J,Gutmann L,Boeve BF,Boylan K,Stoessl AJ,Ross OA,Maragakis NJ,Van Gerpen J,Gerstenhaber M,Gwinn K,Dawson TM,Isacson O,Marder KS,Clark LN,Przedborski SE,Finkbeiner S,Rothstein JD,Wszolek ZK,Rossor MN,Hardy J (2012) Creation of an open-access, mutation-defined fibroblast resource for neurological disease research. PLoS One, 7(8), e43099. 10.1371/journal.pone.0043099.
  2. 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.
  3. Devine MJ,Kaganovich A,Ryten M,Mamais A,Trabzuni D,Manzoni C,McGoldrick P,Chan D,Dillman A,Zerle J,Horan S,Taanman JW,Hardy J,Marti-Masso JF,Healy D,Schapira AH,Wolozin B,Bandopadhyay R,Cookson MR,van der Brug MP,Lewis PA (2012) Correction: Pathogenic LRRK2 Mutations Do Not Alter Gene Expression in Cell Model Systems or Human Brain Tissue. PLoS One, 7(1). 10.1371/annotation/c12e4f9e-5aae-424b-a69f-fddf16976dc5.
  4. Pitceathly RDS,Fassone E,Taanman JW,Sadowski M,Fratter C,Mudanohwo EE,Woodward CE,Sweeney MG,Holton JL,Hanna MG,Rahman S (2011) KEARNS-SAYRE SYNDROME CAUSED BY MUTATIONS IN THE NUCLEAR RRM2B GENE JOURNAL OF INHERITED METABOLIC DISEASE, 34, S158 - S158.
  5. Fassone E,Duncan AJ,Taanman JW,Pagnamenta AT,Sadowski MI,Holand T,Qasim W,Rutland P,Calvo SE,Mootha VK,Bitner-Glindzicz M,Rahman S (2011) BIOCHEMICAL AND MOLECULAR ANALYSIS IN MITOCHONDRIAL COMPLEX I DEFICIENT CHILDREN JOURNAL OF INHERITED METABOLIC DISEASE, 34, S162 - S162.
  6. Gurusamy KS,Farooqui N,Loizidou M,Dijk S,Taanman JW,Whiting S,Farquharson MJ,Fuller BJ,Davidson BR (2011) Influence of zinc and zinc chelator on HT-29 colorectal cell line. Biometals, 24(1), 143 - 151. 10.1007/s10534-010-9382-5.
  7. Fassone E,Duncan AJ,Taanman JW,Pagnamenta AT,Sadowski MI,Holand T,Qasim W,Rutland P,Calvo SE,Mootha VK,Bitner-Glindzicz M,Rahman S (2011) FOXRED1 mutations are a novel cause of mitochondrial complex I deficiency NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 37, 37 - 37.
  8. Pitceathly RD,Fassone E,Taanman JW,Sadowski M,Fratter C,Mudanohwo EE,Woodward CE,Sweeney MG,Holton JL,Hanna MG,Rahman S (2011) Kearns-Sayre syndrome caused by defective R1/p53R2 assembly. J Med Genet, 48(9), 610 - 617. 10.1136/jmg.2010.088328.
  9. Fassone E,Duncan AJ,Taanman JW,Pagnamenta AT,Sadowski M,Holand T,Qasim W,Rutland P,Calvo SE,Mootha VK,Bitner-Glindzicz M,Rahman S (2011) Mutations in the novel chaperone FOXRED1 cause mitochondrial complex I deficiency NEUROMUSCULAR DISORDERS, 21, S19 - S19.
  10. Taanman JW,Rahman S,Clayton P,Leonard JV,King R,Orth M (2011) Mitochondrial respiratory chain enzyme deficiency expressed during muscle development NEUROMUSCULAR DISORDERS, 21, S23 - S23.
  11. Devine MJ,Kaganovich A,Ryten M,Mamais A,Trabzuni D,Manzoni C,McGoldrick P,Chan D,Dillman A,Zerle J,Horan S,Taanman JW,Hardy J,Marti-Masso JF,Healy D,Schapira AH,Wolozin B,Bandopadhyay R,Cookson MR,van der Brug MP,Lewis PA (2011) Pathogenic LRRK2 mutations do not alter gene expression in cell model systems or human brain tissue. PLoS One, 6(7), e22489. 10.1371/journal.pone.0022489.
  12. Devine MJ,Ryten M,Vodicka P,Thomson AJ,Burdon T,Houlden H,Cavaleri F,Nagano M,Drummond NJ,Taanman JW,Schapira AH,Gwinn K,Hardy J,Lewis PA,Kunath T (2011) Parkinson's disease induced pluripotent stem cells with triplication of the α-synuclein locus. Nat Commun, 2, 440. 10.1038/ncomms1453.
  13. Pitceathly RDS,Fassone E,Taanman JW,Sadowski M,Fratter C,Mudanohwo EE,Woodward CE,Sweeney MG,Holton JL,Hanna MG,Rahman S (2011) Kearns-Sayre syndrome caused by defective R1/p53R2 assembly J MED GENET, 48(9), 610 - 617. 10.1136/jmg.2010.088328.
  14. Fassone E,Taanman JW,Duncan A,Rahman S (2011) 14 Mitochondrial cardiomyopathy caused by defective assembly of respiratory chain complex I. Heart, 97(24), e8. 10.1136/heartjnl-2011-301156.14.
  15. Casper CJ,Warner TT,Taanman JW,Nathwani AC (2011) Generation of iPS-derived dopaminergic neurons as an in-vitro model of Rapid-Onset Dystonia Parkinsonism HUMAN GENE THERAPY, 22(10), A42 - A43.
  16. Fassone E,Taanman JW,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. J Med Genet, 48(10), 691 - 697. 10.1136/jmedgenet-2011-100340.
  17. Hernandez MA,Schulz R,Chaplin T,Young BD,Perrett D,Champion MP,Taanman JW,Fensom A,Marinaki AM (2010) The diagnosis of inherited metabolic diseases by microarray gene expression profiling. Orphanet J Rare Dis, 5, 34. 10.1186/1750-1172-5-34.
  18. Gurusamy KS,Farooqui N,Loizidou M,Dijk S,Taanman JW,Whiting S,Farquharson MJ,Fuller BJ,Davidson BR (2010) Influence of zinc and zinc chelator on HT-29 colorectal cell line BRITISH JOURNAL OF SURGERY, 97, 45 - 46.
  19. Fassone E,Duncan AJ,Taanman JW,Pagnamenta AT,Sadowski MI,Holand T,Qasim W,Rutland P,Calvo SE,Mootha VK,Bitner-Glindzicz M,Rahman S (2010) FOXRED1, encoding an FAD-dependent oxidoreductase complex-I-specific molecular chaperone, is mutated in infantile-onset mitochondrial encephalopathy. Hum Mol Genet, 19(24), 4837 - 4847. 10.1093/hmg/ddq414.
  20. Gegg ME,Cooper JM,Chau KY,Rojo M,Schapira AH,Taanman JW (2010) Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy. Hum Mol Genet, 19(24), 4861 - 4870. 10.1093/hmg/ddq419.
  21. Taanman JW,Heiske M,Letellier T (2010) Measurement of kinetic parameters of human platelet DNA polymerase gamma. Methods, 51(4), 374 - 378. 10.1016/j.ymeth.2010.03.002.
  22. Ginsberg L,Taanman JW (2010) POG01 Anti-thymidine phosphorylase antibodies in the diagnosis of mitochondrial neurogastrointestinal encephalomyopathy. J Neurol Neurosurg Psychiatry, 81(11), e48. 10.1136/jnnp.2010.226340.123.
  23. Hafez TS,Glantzounis GK,Fusai G,Taanman JW,Wignarajah P,Parkes H,Fuller B,Davidson BR,Seifalian AM (2010) Intracellular oxygenation and cytochrome oxidase C activity in ischemic preconditioning of steatotic rabbit liver. Am J Surg, 200(4), 507 - 518. 10.1016/j.amjsurg.2009.09.028.
  24. Ruhanen H,Borrie S,Szabadkai G,Tyynismaa H,Jones AWE,Kang D,Taanman J-W,Yasukawa T (2010) Mitochondrial single-stranded DNA binding protein is required for maintenance of mitochondrial DNA and 7S DNA but is not required for mitochondrial nucleoid organization. Biochim Biophys Acta., 1803(8), 931 - 939. 10.1016/j.bbamcr.2010.04.008.
  25. Sagar J,Sales K,Taanman JW,Dijk S,Winslet M (2010) Lowering the apoptotic threshold in colorectal cancer cells by targeting mitochondria. Cancer Cell Int, 10, 31. 10.1186/1475-2867-10-31.
  26. Taanman JW,Rahman S,Pagnamenta AT,Morris AA,Bitner-Glindzicz M,Wolf NI,Leonard JV,Clayton PT,Schapira AH (2009) Analysis of mutant DNA polymerase gamma in patients with mitochondrial DNA depletion Hum.Mutat., 30(2), 248 - 254.
  27. Taanman JW,Daras M,Albrecht J,Davie CA,Mallam EA,Muddle JR,Weatherall M,Warner TT,Schapira AH,Ginsberg L (2009) Characterization of a novel TYMP splice site mutation associated with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) Neuromuscul.Disord., 19(2), 151 - 154.
  28. Gegg ME,Cooper JM,Schapira AH,Taanman JW (2009) Silencing of PINK1 expression affects mitochondrial DNA and oxidative phosphorylation in dopaminergic cells PLoS.One., 4(3), e4756.
  29. Grunewald A,Gegg ME,Taanman JW,King RH,Klein C,Schapira A (2009) Differential Effects of PINK1 Nonsense and Missense Mutations on Mitochondrial Function and Morphology NEUROLOGY, 72(11), A388 - A388.
  30. Bradley LJ,Taanman JW,Kallis C,Orrell RW (2009) Increased sensitivity of myoblasts to oxidative stress in amyotrophic lateral sclerosis peripheral tissues Experimental Neurology, 218(1), 92 - 97. 10.1016/j.expneurol.2009.04.007.
  31. Sagar J,Sales K,Dijk S,Taanman J,Seifalian A,Winslet M (2009) Does doxycycline work in synergy with cisplatin and oxaliplatin in colorectal cancer? World J Surg Oncol, 7, 2. 10.1186/1477-7819-7-2.
  32. Sagar J,Sales K,Dijk S,Taanman J,Seifalian A,Winslet M (2009) Does Doxycycline work in synergy with cisplatin and oxaliplatin in colorectal cancer? WORLD J SURG ONCOL, 7. 10.1186/1477-7819-7-2.
  33. Wolf NI,Rahman S,Schmitt B,Taanman JW,Duncan AJ,Harting I,Wohlrab G,Ebinger F,Rating D,Bast T (2009) Status epilepticus in children with Alpers' disease caused by POLG1 mutations: EEG and MRI features. Epilepsia, 50(6), 1596 - 1607. 10.1111/j.1528-1167.2008.01877.x.
  34. Grunewald A,Gegg ME,Taanman JW,King RH,Kock N,Klein C,Schapira AH (2009) Differential effects of PINK1 nonsense and missense mutations on mitochondrial function and morphology Exp.Neurol., 219(1), 266 - 273.
  35. Poulton J,Hirano M,Spinazzola A,Arenas Hernandez M,Jardel C,Lombès A,Czermin B,Horvath R,Taanman JW,Rotig A,Zeviani M,Fratter C (2009) Collated mutations in mitochondrial DNA (mtDNA) depletion syndrome (excluding the mitochondrial gamma polymerase, POLG1). Biochim Biophys Acta, 1792(12), 1109 - 1112. 10.1016/j.bbadis.2009.08.016.
  36. Rocher C,Taanman JW,Pierron D,Faustin B,Benard G,Rossignol R,Malgat M,Pedespan L,Letellier T (2008) Influence of mitochondrial DNA level on cellular energy metabolism: implications for mitochondrial diseases. J Bioenerg Biomembr, 40(2), 59 - 67. 10.1007/s10863-008-9130-5.
  37. Ginsberg L,Taanman JW (2008) Anti-thymidine phosphorylase antibodies in the diagnosis of mitochondrial neurogastrointestinal encephalomyopathy EUROPEAN JOURNAL OF NEUROLOGY, 15, 183 - 183.
  38. Misbahuddin A,Placzek M,Lennox G,Taanman JW,Warner TT (2007) Myoclonus-dystonia syndrome with severe depression is caused by an exon-skipping mutation in the epsilon-sarcoglycan gene Movement Disorders, 22(8), 1173 - 1175.
  39. Mousson de Camaret B,Taanman JW,Padet S,Chassagne M,Mayencon M,Clerc-Renaud P,Mandon G,Zabot MT,Lachaux A,Bozon D (2007) Kinetic properties of mutant deoxyguanosine kinase in a case of reversible hepatic mtDNA depletion. Biochemical Journal, 402(2), 377 - 385. 10.1042/BJ20060705.
  40. Ginsberg L,Schapira AH,Taanman JW (2007) Relapsing neuropathy in an 18-year-old woman. The Lancet Neurology, 6(2), 192 - 198.
  41. Ginsberg L,Daras M,Davie C,Albrecht J,Schapira AHV,Weatherall M,Taanman JW (2007) A novel MNGIE mutation EUROPEAN JOURNAL OF NEUROLOGY, 14, 238 - 238.
  42. Ginsberg L,Taanman JW (2006) MNGIE neuropathy mimicking Guillain-Barre syndrome JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 77(12), 1400 - 1400.
  43. Pagnamenta AT,Taanman JW,Wilson CJ,Anderson NE,Marotta R,Duncan AJ,Bitner-Glindzicz M,Taylor RW,Laskowski A,Thorburn DR,Rahman S (2006) Dominant inheritance of premature ovarian failure associated with mutant mitochondrial DNA polymerase gamma Human Reproduction, 21(10), 2467 - 2473. 10.1093/humrep/del076.
  44. Pagnamenta AT,Hargreaves IP,Duncan AJ,Taanman JW,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(3), 214 - 221. 10.1016/j.ymgme.2006.05.003.
  45. Taanman J-W,Williams SL (2005) The human mitochondrial genome: mechanisms of expression and maintenance, 95 - 246.
  46. Labarthe F,Dobbelaere D,Devisme L,De Muret A,Jardel C,Taanman JW,Gottrand F,Lonmbes A (2005) CLINICAL, BIOCHEMICAL AND MORPHOLOGICAL FEATURES OF HEPATOCEREBRAL SYNDROME WITH MITOCHONDRIAL DNA DEPLETION DUE TO DEOXYGUANOSINE KINASE DEFICIENCY J INHERIT METAB DIS, 28, 128 - 128.
  47. Taanman JW,Schapira AHV (2005) Analysis of the trinucleotide CAG repeat from the DNA polymerase gamma gene (POLG) in patients with Parkinson's disease Neuroscience Letters, 376(1), 56 - 59.
  48. Labarthe F,Dobbelaere D,Devisme L,De Muret A,Jardel C,Taanman JW,Gottrand F,Lombes A (2005) Clinical, biochemical and morphological features of hepatocerebral syndrome with mitochondrial DNA depletion due to deoxyguanosine kinase deficiency. Journal of Hepatology, 43(2), 333 - 341.
  49. Proukakis C,Cooper JM,Taanman JW,Warner TT (2005) Cellular studies of spastin reveal no gain of function, and suggest translation from the 2nd ATG EUR J NEUROL, 12, 323 - 323.
  50. Lo S,Tolner B,Taanman JW,Cooper JM,Gu M,Hartley JA,Schapira AH,Hochhauser D (2005) Assessment of the significance of mitochondrial DNA damage by chemotherapeutic agents. International Journal of Oncology, 27(2), 337 - 344.

Qualifications

  • 1991: Doctor of Philosophy, To be updated
  • 1985: Masters, To be updated
  • 1982: Bachelor of Science, To be updated