Research Focus


  • Mitochondrial Disease
  • Drosophila Genetics
  • Genetic Disorders
  • Embryogenesis
  • Transcription
  • Stem Cells & Development
  • Neural Stem Cells
  • Single Cell Sequencing
  • Mitochondria
  • Neurology
  • Clinical Conditions

  • Genetic disorders
  • Equipment & Techniques

  • Other
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    Dr Jelle van den Ameele

    University Position
    Wellcome Career Development Fellow
    Dr Jelle van den Ameele is pleased to consider applications from prospective PhD students.


    I am a neurologist with an interest in developmental neurobiology and mitochondrial genetics. In the lab, we study how cells and tissues respond to mitochondrial dysfunction, focusing on neural stem cells and the brain. Our goal is to identify novel determinants of tissue-specific phenotypes of mitochondrial and neurodegenerative diseases and to uncover potential therapeutic targets to treat these disorders. We use a wide range of techniques and model systems, but mostly take advantage of the powerful genetics of Drosophila. We combine targeted genetic manipulations with confocal and super-resolution imaging, biosensors, and innovative DamID-based sequencing approaches to study the metabolism, transcriptional and epigenetic changes of different cell-types within the brain of a living organism affected by mitochondrial dysfunction. I also participate in the clinical research programme related to the mitochondrial genetics clinic MitoCamb ( in Addenbrooke's CUH NHS Trust.

    Key Publications

    Opportunities for mitochondrial disease gene therapy.

    Journal: Nat Rev Drug Discov
    E-pub date: 1 Jun 2023
    Authors: C Viscomi, J van den Ameele, KC Meyer, PF Chinnery

    Mitochondrial respiration and dynamics of in vivo neural stem cells.

    Journal: Development
    E-pub date: 1 Dec 2022
    Authors: S Petridi, D Dubal, R Rikhy, J van den Ameele

    Reduced chromatin accessibility correlates with resistance to Notch activation.

    Journal: Nature Communications
    E-pub date: 25 Apr 2022
    Authors: J van den Ameele, R Krautz, SW Cheetham, APA Donovan, O Llorà-Batlle, R Yakob, AH Brand

    BCL6 controls neurogenesis through Sirt1-dependent epigenetic repression of selective Notch targets.

    Journal: Nat Neurosci
    E-pub date: 1 Dec 2012
    Authors: L Tiberi, J van den Ameele, J Dimidschstein, J Piccirilli, D Gall, A Herpoel, A Bilheu, J Bonnefont, M Iacovino, M Kyba, T Bouschet, P Vanderhaeghen

    An intrinsic mechanism of corticogenesis from embryonic stem cells.

    Journal: Nature
    E-pub date: 18 Sep 2008
    Authors: N Gaspard, T Bouschet, R Hourez, J Dimidschstein, G Naeije, J van den Ameele, I Espuny-Camacho, A Herpoel, L Passante, SN Schiffmann, A Gaillard, P Vanderhaeghen


    Multisystem pathology in McLeod syndrome.

    Journal: Neuropathology
    E-pub date: 1 Apr 2024
    Authors: KR Schon, DG O'Donovan, M Briggs, JB Rowe, L Wijesekera, PF Chinnery, J van den Ameele

    An oligodendrocyte silencer element underlies the pathogenic impact of lamin B1 structural variants.

    E-pub date: 9 Aug 2023
    Authors: B Nmezi, GR Bey, TD Oranburg, K Dudnyk, SM Lardo, N Herdman, A Jacko, S Rubio, EL Alcocer, J Kofler, D Kim, J Rankin, E Kivuva, N Gutowski, K Schon, J van den Ameele, PF Chinnery, SB Sousa, F Palavra, C Toro, F Pinto E Vairo, J Saute, L Pan, M Alturkustani, R Hammond, F Gros-Louis, M Gold, Y Park, G Bernard, R Raininko, J Zhou, SJ Hainer, QS Padiath

    In preprints: releasing the brakes on neuronal maturation.

    Journal: Development
    E-pub date: 15 Dec 2022
    Authors: T Rayon, J van den Ameele

    SOX9 maintains human foetal lung tip progenitor state by enhancing WNT and RTK signalling.

    Journal: EMBO J
    E-pub date: 2 Nov 2022
    Authors: D Sun, O Llora Batlle, J van den Ameele, JC Thomas, P He, K Lim, W Tang, C Xu, KB Meyer, SA Teichmann, JC Marioni, SP Jackson, AH Brand, EL Rawlins

    An organoid CRISPRi screen revealed that SOX9 primes human fetal lung tip progenitors to receive WNT and RTK signals

    E-pub date: 1 Aug 2022
    Authors: D Sun, OL Batlle, J van den Ameele, J Thomas, P He, K Lim, W Tang, C Xu, K Meyer, S Teichmann, J Marioni, S Jackson, A Brand, E Rawlins

    In vivo targeted DamID identifies CHD8 genomic targets in fetal mouse brain.

    Journal: iScience
    E-pub date: 19 Nov 2021
    Authors: AA Wade, J van den Ameele, SW Cheetham, R Yakob, AH Brand, AS Nord

    MitoPhen database: a human phenotype ontology-based approach to identify mitochondrial DNA diseases.

    Journal: Nucleic Acids Res
    E-pub date: 27 Sep 2021
    Authors: TE Ratnaike, D Greene, W Wei, A Sanchis-Juan, KR Schon, J van den Ameele, L Raymond, R Horvath, E Turro, PF Chinnery

    Metabolic decisions in development and disease.

    Journal: Development
    E-pub date: 1 Jun 2021
    Authors: L Mosteiro, H Hariri, J van den Ameele

    [11C]PK11195-PET Brain Imaging of the Mitochondrial Translocator Protein in Mitochondrial Disease.

    Journal: Neurology
    E-pub date: 31 May 2021
    Authors: J van den Ameele, Y Hong, R Manavaki, A Kouli, H Biggs, Z MacIntyre, R Horvath, P Yu-Wai-Man, E Reid, CH Williams-Gray, E Bullmore, F Aigbirhio, T Fryer, P Chinnery

    Mitochondrial Diseases: A Diagnostic Revolution.

    Journal: Trends Genet
    E-pub date: 1 Sep 2020
    Authors: KR Schon, T Ratnaike, J van den Ameele, R Horvath, PF Chinnery

    Chronic pain is common in mitochondrial disease.

    Journal: Neuromuscul Disord
    E-pub date: 1 May 2020
    Authors: J van den Ameele, J Fuge, RDS Pitceathly, S Berry, Z McIntyre, MG Hanna, M Lee, PF Chinnery

    Mitochondrial heteroplasmy beyond the oocyte bottleneck.

    Journal: Semin Cell Dev Biol
    E-pub date: 1 Jan 2020
    Authors: J van den Ameele, AYZ Li, H Ma, PF Chinnery

    Epigenetic remodelling licences adult cholangiocytes for organoid formation and liver regeneration.

    Journal: Nat Cell Biol
    E-pub date: 1 Nov 2019
    Authors: L Aloia, MA McKie, G Vernaz, L Cordero-Espinoza, N Aleksieva, J van den Ameele, F Antonica, B Font-Cunill, A Raven, R Aiese Cigliano, G Belenguer, RL Mort, AH Brand, M Zernicka-Goetz, SJ Forbes, EA Miska, M Huch

    Cortical Neurogenesis Requires Bcl6-Mediated Transcriptional Repression of Multiple Self-Renewal-Promoting Extrinsic Pathways.

    Journal: Neuron
    E-pub date: 25 Sep 2019
    Authors: J Bonnefont, L Tiberi, J van den Ameele, D Potier, ZB Gaber, X Lin, A Bilheu, A Herpoel, FD Velez Bravo, F Guillemot, S Aerts, P Vanderhaeghen

    TaDa! Analysing cell type-specific chromatin in vivo with Targeted DamID.

    Journal: Curr Opin Neurobiol
    E-pub date: 1 Jun 2019
    Authors: J van den Ameele, R Krautz, AH Brand

    Targeted DamID reveals differential binding of mammalian pluripotency factors.

    Journal: Development
    E-pub date: 17 Oct 2018
    Authors: SW Cheetham, WH Gruhn, J van den Ameele, R Krautz, TD Southall, T Kobayashi, MA Surani, AH Brand

    High prevalence of focal and multi-focal somatic genetic variants in the human brain.

    Journal: Nat Commun
    E-pub date: 15 Oct 2018
    Authors: MJ Keogh, W Wei, J Aryaman, L Walker, J van den Ameele, J Coxhead, I Wilson, M Bashton, J Beck, J West, R Chen, C Haudenschild, G Bartha, S Luo, CM Morris, NS Jones, J Attems, PF Chinnery

    Teenage-onset progressive myoclonic epilepsy due to a familial C9orf72 repeat expansion.

    Journal: Neurology
    E-pub date: 20 Feb 2018
    Authors: J van den Ameele, I Jedlickova, A Pristoupilova, A Sieben, S Van Mossevelde, C Ceuterick-de Groote, H Hůlková, R Matej, A Meurs, C Van Broeckhoven, SF Berkovic, P Santens, S Kmoch, B Dermaut

    A BCL6/BCOR/SIRT1 Complex Triggers Neurogenesis and Suppresses Medulloblastoma by Repressing Sonic Hedgehog Signaling (vol 26, pg 797, 2012)

    Journal: CANCER CELL
    E-pub date: 1 Aug 2015
    Authors: L Tiberi, J Bonnefont, J van den Ameele, S-D Le Bon, A Herpoel, A Bilheu, BW Baron, P Vanderhaeghen

    A BCL6/BCOR/SIRT1 complex triggers neurogenesis and suppresses medulloblastoma by repressing Sonic Hedgehog signaling.

    Journal: Cancer Cell
    E-pub date: 8 Dec 2014
    Authors: L Tiberi, J Bonnefont, J van den Ameele, S-D Le Bon, A Herpoel, A Bilheu, BW Baron, P Vanderhaeghen

    A familial heterozygous null mutation of MET in autism spectrum disorder.

    Journal: Autism Res
    E-pub date: 1 Oct 2014
    Authors: N Lambert, V Wermenbol, B Pichon, S Acosta, J van den Ameele, C Perazzolo, D Messina, M-F Musumeci, B Dessars, A De Leener, M Abramowicz, C Vilain

    Thinking out of the dish: what to learn about cortical development using pluripotent stem cells.

    Journal: Trends Neurosci
    E-pub date: 1 Jun 2014
    Authors: J van den Ameele, L Tiberi, P Vanderhaeghen, I Espuny-Camacho

    Ephrin-B1 controls the columnar distribution of cortical pyramidal neurons by restricting their tangential migration.

    Journal: Neuron
    E-pub date: 18 Sep 2013
    Authors: J Dimidschstein, L Passante, A Dufour, J van den Ameele, L Tiberi, T Hrechdakian, R Adams, R Klein, DC Lie, Y Jossin, P Vanderhaeghen

    Eomesodermin induces Mesp1 expression and cardiac differentiation from embryonic stem cells in the absence of Activin.

    Journal: EMBO Rep
    E-pub date: 1 Apr 2012
    Authors: J van den Ameele, L Tiberi, A Bondue, C Paulissen, A Herpoel, M Iacovino, M Kyba, C Blanpain, P Vanderhaeghen

    Cortical neurogenesis and morphogens: diversity of cues, sources and functions.

    Journal: Curr Opin Cell Biol
    E-pub date: 1 Apr 2012
    Authors: L Tiberi, P Vanderhaeghen, J van den Ameele

    Late-onset post-irradiation vasculopathy of the posterior cerebral vasculature.

    Journal: Acta Neurol Belg
    E-pub date: 1 Mar 2012
    Authors: J van den Ameele, A Sieben, C Van den Broecke, T Boterberg, L Defreyne, E Achten, M Lammens, D Hemelsoet

    Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).

    Journal: Brain
    E-pub date: 1 Sep 2010
    Authors: SJ Pittock, J Debruyne, KN Krecke, C Giannini, J van den Ameele, V De Herdt, A McKeon, RD Fealey, BG Weinshenker, AJ Aksamit, BR Krueger, EA Shuster, BM Keegan

    Generation of cortical neurons from mouse embryonic stem cells.

    Journal: Nat Protoc
    E-pub date: 1 Aug 2009
    Authors: N Gaspard, T Bouschet, A Herpoel, G Naeije, J van den Ameele, P Vanderhaeghen