Department

Institute

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Research Theme Lifelong Brain Development and Brain Ageing Beyond the Neuron

Research Focus

Keywords

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

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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.

Interests

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 (https://www-neurosciences.medschl.cam.ac.uk/mitocamb/) in Addenbrooke's CUH NHS Trust.

Key Publications

Opportunities for mitochondrial disease gene therapy.

DOI: http://doi.org/10.1038/d41573-023-00067-z
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.

DOI: http://doi.org/10.1242/dev.200870
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.

DOI: http://doi.org/10.1038/s41467-022-29834-z
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

Mitochondrial Diseases: A Diagnostic Revolution.

DOI: http://doi.org/10.1016/j.tig.2020.06.009
Journal: Trends Genet
E-pub date: 1 Sep 2020
Authors: KR Schon, T Ratnaike, J van den Ameele, R Horvath, PF Chinnery

Neural stem cell temporal patterning and brain tumour growth rely on oxidative phosphorylation.

DOI: http://doi.org/10.7554/eLife.47887
Journal: Elife
E-pub date: 12 Sep 2019
Authors: J van den Ameele, AH Brand

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

DOI: http://doi.org/10.1038/nn.3264
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.

DOI: http://doi.org/10.1038/nature07287
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

Publications

Multisystem pathology in McLeod syndrome.

DOI: http://doi.org/10.1111/neup.12935
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.

DOI: http://doi.org/10.1101/2023.08.03.551473
Journal:
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

Case report: Mutations in DNAJC30 causing autosomal recessive Leber hereditary optic neuropathy are common amongst Eastern European individuals.

DOI: http://doi.org/10.3389/fneur.2023.1292320
Journal: Front Neurol
E-pub date: 1 Aug 2023
Authors: TC Major, ES Arany, K Schon, M Simo, V Karcagi, J van den Ameele, P Yu Wai Man, PF Chinnery, C Olimpio, R Horvath

In preprints: releasing the brakes on neuronal maturation.

DOI: http://doi.org/10.1242/dev.201477
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.

DOI: http://doi.org/10.15252/embj.2022111338
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

DOI: http://doi.org/10.1101/2022.01.27.478034
Journal:
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

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

DOI: http://doi.org/10.1212/WNL.0000000000012559
Journal: Neurology
E-pub date: 7 Dec 2021
Authors:

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

DOI: http://doi.org/10.1016/j.isci.2021.103234
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.

DOI: http://doi.org/10.1093/nar/gkab726
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.

DOI: http://doi.org/10.1242/dev.199609
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.

DOI: http://doi.org/10.1212/WNL.0000000000012033
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

Chronic pain is common in mitochondrial disease.

DOI: http://doi.org/10.1016/j.nmd.2020.02.017
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.

DOI: http://doi.org/10.1016/j.semcdb.2019.10.001
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.

DOI: http://doi.org/10.1038/s41556-019-0402-6
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.

DOI: http://doi.org/10.1016/j.neuron.2019.06.027
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.

DOI: http://doi.org/10.1016/j.conb.2019.01.021
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.

DOI: http://doi.org/10.1242/dev.170209
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.

DOI: http://doi.org/10.1038/s41467-018-06331-w
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.

DOI: http://doi.org/10.1212/WNL.0000000000004999
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)

DOI: http://doi.org/10.1016/j.ccell.2014.12.011
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.

DOI: http://doi.org/10.1016/j.ccell.2014.10.021
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.

DOI: http://doi.org/10.1002/aur.1396
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.

DOI: http://doi.org/10.1016/j.tins.2014.03.005
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.

DOI: http://doi.org/10.1016/j.neuron.2013.07.015
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.

DOI: http://doi.org/10.1038/embor.2012.23
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.

DOI: http://doi.org/10.1016/j.ceb.2012.01.010
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.

DOI: http://doi.org/10.1007/s13760-012-0014-4
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).

DOI: http://doi.org/10.1093/brain/awq164
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.

DOI: http://doi.org/10.1038/nprot.2009.157
Journal: Nat Protoc
E-pub date: 1 Aug 2009
Authors: N Gaspard, T Bouschet, A Herpoel, G Naeije, J van den Ameele, P Vanderhaeghen