Dr Jelle van den Ameele University positionWellcome Clinical Research Career Development Fellow Dr Jelle van den Ameele is pleased to consider applications from prospective PhD students. DepartmentsDepartment of Clinical Neurosciences InstitutesMitochondrial Biology Unit, MRC Home pagehttps://www.mrc-mbu.cam.ac.uk/researc... (personal home page) Research ThemesInterestsI 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 in Addenbrooke’s. Research Focus
EquipmentConfocal microscopy Drosophila genetics In utero electroporation Live imaging Mouse genetics Targeted DamID, DamID-seq CollaboratorsNo collaborators listed Associated News ItemsPublications2022Petridi S, Dubal D, Rikhy R, van den Ameele J (2022), “Mitochondrial respiration and dynamics of in vivo neural stem cells” Development 149(23):dev200870. doi: 10.1242/dev.200870. Rayon T, van den Ameele J (2022), “In preprints: releasing the brakes on neuronal maturation” Development 149(24):dev201477. doi: 10.1242/dev.201477. Sun D, Llora Batlle O, van den Ameele J, Thomas JC, He P, Lim K, Tang W, Xu C, Meyer KB, Teichmann SA, Marioni JC, Jackson SP, Brand AH, Rawlins EL (2022), “SOX9 maintains human foetal lung tip progenitor state by enhancing WNT and RTK signalling” EMBO J 41(21):e111338. doi: 10.15252/embj.2022111338 van den Ameele J, Krautz R, Cheetham SW, Donovan APA, Llorà-Batlle O, Yakob R, Brand AH (2022), “Reduced chromatin accessibility correlates with resistance to Notch activation” Nat Commun 13(1):2210. doi: 10.1038/s41467-022-29834-z. 2021Mosteiro L, Hariri H, van den Ameele J (2021), “Metabolic decisions in development and disease” Development 148: dev199609 Ratnaike T, Greene D, Wei W, Sanchis-Juan A, Schon K, van den Ameele J, Raymond L, Horvath R, Turro E, Chinnery PF (2021), “MitoPhen database: a Human Phenotype Ontology-based approach to identify mitochondrial DNA diseases” Nucleic Acids Res gkab726 van den Ameele J, Hong YT, Manavaki R, Kouli A, Biggs H, McIntyre Z, Horvath R, Yu-Wai-Man P, Reid E, Williams-Gray CG, Bullmore ET, Aigbirhio FI, Fryer TD, Chinnery PF (2021), “[11C]PK11195-PET Brain Imaging of the Mitochondrial Translocator Protein in Mitochondrial Disease” Neurology doi: 10.1212/WNL.0000000000012033 Wade AA*, van den Ameele J*, Cheetham SW, Yakob R, Brand AH, Nord AS (2021), “In vivo Targeted DamID identifies CHD8 genomic targets in fetal mouse brain” iScience 24(11):103234 2020Schon K, Ratnaike T, van den Ameele J, Horvath R, Chinnery PF (2020), “Mitochondrial diseases: a diagnostic revolution” Trends Genet 36(9):702-717. doi: 10.1016/j.tig.2020.06.009 van den Ameele J*, Fuge J*, Pitceathly RDS, Berry S, McIntyre Z, Hanna MG, Lee M, Chinnery PF (2020), “Chronic pain is common in mitochondrial disease” Neuromuscul Disord May;30(5):413-419. doi: 10.1016/j.nmd.2020.02.017. van den Ameele J, Li AYZ, Ma H, Chinnery PF (2020), “Mitochondrial heteroplasmy beyond the oocyte bottleneck” Semin Cell Dev Biol Jan;97:156-166. doi: 10.1016/j.semcdb.2019.10.001. 2019Aloia L, McKie MA, Vernaz G, Cordero-Espinoza L, Aleksieva N, van den Ameele J, Antonica F, Font-Cunill B, Raven A, Aiese Cigliano R, Belenguer G, Mort RL, Brand AH, Zernicka-Goetz M, Forbes SJ, Miska EA, Huch M (2019), “Epigenetic remodelling licences adult cholangiocytes for organoid formation and liver regeneration” Nat Cell Biol Nov;21(11):1321-1333. doi: 10.1038/s41556-019-0402-6. Bonnefont J, Tiberi L, van den Ameele J, Potier D, Gaber ZB, Lin X, Bilheu A, Herpoel A, Velez Bravo FD, Guillemot F, Aerts S, Vanderhaeghen P (2019), “Cortical Neurogenesis Requires Bcl6-Mediated Transcriptional Repression of Multiple Self-Renewal-Promoting Extrinsic Pathways” Neuron Sep 25;103(6):1096-1108.e4. doi: 10.1016/j.neuron.2019.06.027. van den Ameele J*, Krautz R*, Brand AH (2019), “TaDa! Analyzing cell-type specific chromatin in vivo with Targeted DamID” Curr Opin Neurobiol Jun;56:160-166. doi: 10.1016/j.conb.2019.01.021. van den Ameele J, Brand AH (2019), “Neural stem cell temporal patterning and brain tumour growth rely on oxidative phosphorylation” eLife 8:e47887. doi: 10.7554/eLife.47887 2018Cheetham SW*, Gruhn WH*, van den Ameele J*, Krautz R*, Southall TD, Kobayashi T, Surani MA, Brand AH. (2018), “Targeted DamID reveals differential binding of mammalian pluripotency factors. ” Development Oct 17;145(20):dev170209. doi: 10.1242/dev.170209. Keogh MJ, Wei W, Aryaman J, Walker L, van den Ameele J, Coxhead J, Wilson I, Bashton M, Beck J, West J, Chen R, Haudenschild C, Bartha G, Luo S, Morris CM, Jones NJ, Attems J, Chinnery PF. (2018), “High prevalence of focal and multi-focal somatic genetic variants in the human brain. ” Nat Commun Oct 15;9(1):4257. doi: 10.1038/s41467-018-06331-w. van den Ameele J*, Jedlickova I*, Pristoupilova A, Sieben A, Van Mossevelde S, Ceuterick-de Groote C, Hulkova H, Matej R, Meurs A, Van Broeckhoven C, Berkovic SF, Santens P, Kmoch S, Dermaut B (2018), “Teenage-onset progressive myoclonic epilepsy due to a familial C9orf72 repeat expansion” Neurology 90 (8) 2014Lambert N, Wermenbol L, Pichon B, Acosta S, van den Ameele J, Perazzolo C, Messina D, Musumeci F, Dessars B, De Leener A, Abramowicz M, Vilain C (2014), “A familial heterozygous null mutation of MET in autism spectrum disorder” Autism Res 7(5):617-22 Tiberi L, Bonnefont J, van den Ameele J, Le Bon SD, Herpoel A, Bilheu A, Baron BW, Vanderhaeghen P (2014), “A Bcl6/BCoR/Sirt1 complex Triggers Neurogenesis and Suppresses Medulloblastoma by Repressing Sonic Hedgehog Signalling” Cancer Cell 26(6):797-812 van den Ameele J, Tiberi L, Vanderhaeghen P, Espuny-Camacho I (2014), “Thinking out of the dish: what to learn about cortical development using pluripotent stem cells” Trends Neurosci 37(6):334-42 2013Dimidschstein J, Passante L, Dufour A, van den Ameele J, Tiberi L, Hrechdakian T, Adams R, Klein R, Chichung Lie D, Jossin D, Vanderhaeghen P (2013), “Ephrin-B1 controls the columnar distribution of cortical pyramidal neurons by restricting their tangential migration” Neuron 79(6):1123-35 2012Tiberi L*, van den Ameele J*, Dimidschstein J, Piccirilli J, Gall D, Herpoel A, Bilheu A, Bonnefont J, Iacovino M, Kyba M, Bouschet T, Vanderhaeghen P (2012), “Bcl6 induces neurogenesis through Sirt1-dependent epigenetic repression of selective Notch targets” Nat Neurosci 15(12):1627-35 Tiberi L, Vanderhaeghen P, van den Ameele J (2012), “Cortical Neurogenesis and Morphogens: Diversity of Cues, Sources and Functions” Curr Opin Cell Biol 24(2):269-76 van den Ameele J*, Tiberi L*, Bondue A*, Paulissen C, Herpoel A, Iacovino M, Kyba M, Blanpain C, Vanderhaeghen P (2012), “Eomesodermin induces Mesp1 expression and cardiac differentiation from embryonic stem cells in the absence of Activin” Embo Rep 13(4):355-62 van den Ameele J, Sieben A, Van den Broecke C, Boterberg T, Defreyne L, Achten E, Lammens M, Hemelsoet D (2012), “Late-onset post-irradiation vasculopathy of the posterior cerebral vasculature” Acta Neurol Belg 112(1):101-4 2010Pittock SJ, Debruyne J, Krecke K, Giannini C, van den Ameele J, De Herdt V, McKeon A, Fealey RD, Weinshenker BG, Aksamit AJ, Krueger BR, Shuster EA, Keegan BM (2010), “Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS)” Brain 133(9):2626-34 2009Gaspard N, Bouschet T, Herpoel A, Naeije G, van den Ameele J, Vanderhaeghen P (2009), “Generation of cortical neurons from mouse embryonic stem cells” Nat Protoc 4(10):1454-63 2008Gaspard N, Bouschet T, Hourez R, Dimidschstein J, Naeije G, van den Ameele J, Espuny-Camacho I, Herpoel A, Passante L, Schiffmann SN, Gaillard A, Vanderhaeghen P (2008), “An intrinsic mechanism of corticogenesis from embryonic stem cells” Nature 455(7211):351-7 van den Ameele J, Van Landegem W, Wuyts W, De Bleecker J (2008), “Recurrent post-infectious rhabdomyolysis in muscle CPT-II deficiency caused by a novel missense mutation” Acta Neurol Belg 108(4):155-60 |