Molekulare Ernährung
Forschungsschwerpunkte Infektionsforschung und Typ 1 Diabetes
Forschungsschwerpunkte Neonatologie und Pädiatrische Intensivmedizin
Stammzell Metabolismus
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Molekulare Ernährung
Forschungsschwerpunkte Infektionsforschung und Typ 1 Diabetes
Forschungsschwerpunkte Neonatologie und Pädiatrische Intensivmedizin
Stammzell Metabolismus
Source: Human iPSC-derived cerebral organoid stained with DAPI (blue) and GFAP (red) (image by Werner Dykstra).

Selected publications

  • Tolle I, Tiranti V, Prigione A. Modeling mitochondrial DNA diseases: from base editing to pluripotent stem-cell-derived organoids. EMBO Rep. 2023 Mar 6:e55678. doi: 10.15252/embr.202255678. Online ahead of print. PMID: 36876467
  • Daneshgar N, Leidinger MR, Le S, Hefti M, Prigione A, Dai DF. Activated microglia and neuroinflammation as a pathogenic mechanism in Leigh syndrome. Front Neurosci. 2023 Jan 18;16:1068498. doi: 10.3389/fnins.2022.1068498. eCollection 2022.
  • Raskó T, Pande A, Radscheit K, Zink A, Singh M, Sommer C, Wachtl G, Kolacsek O, Inak G, Szvetnik A, Petrakis S, Bunse M, Bansal V, Selbach M, Orbán TI, Prigione A, Hurst LD, Izsvák Z. A Novel Gene Controls a New Structure: PiggyBac Transposable Element-Derived 1, Unique to Mammals, Controls Mammal-Specific Neuronal Paraspeckles.  Mol Biol Evol. 2022 Oct 7;39(10):msac175. doi: 10.1093/molbev/msac175. PMID: 36205081 Free PMC article.
  • Steiner T, Zink A, Henke MT, Cecchetto G, Buenning M, Rossi A, Schuelke M, Prigione A. RNA-based generation of iPSCs from a boy carrying the mutation m.9185 TC in the mitochondrial gene MT-ATP6 and from his healthy mother.  Stem Cell Res. 2022 Sep 15;64:102920. doi: 10.1016/j.scr.2022.102920. Online ahead of print. PMID: 36137325 Free article.
  • Lickfett S, Menacho C, Zink A, Telugu NS, Beller M, Diecke S, Cambridge S, Prigione A. High-content analysis of neuronal morphology in human iPSC-derived neurons.  STAR Protoc. 2022 Aug 6;3(3):101567. doi: 10.1016/j.xpro.2022.101567. eCollection 2022 Sep 16. PMID: 35990743 Free PMC article.
  • Zink A, Haferkamp U, Wittich A, Beller M, Pless O, Prigione A. High-content screening of mitochondrial polarization in neural cells derived from human pluripotent stem cells.  STAR Protoc. 2022 Aug 5;3(3):101602. doi: 10.1016/j.xpro.2022.101602. eCollection 2022 Sep 16. PMID: 35959496 Free PMC article.
  • Lorenz C, Zink A, Henke MT, Staege S, Mlody B, Bünning M, Wanker E, Diecke S, Schuelke M, Prigione A. Generation of four iPSC lines from four patients with Leigh syndrome carrying homoplasmic mutations m.8993T>G or m.8993T>C in the mitochondrial gene MT-ATP6. Stem Cell Res 2022.
  • Rossi A, Lickfett S, Martins S, Prigione A. A call for consensus guidelines on monitoring the integrity of nuclear and mitochondrial genome in human pluripotent stem cells. Stem Cell Reports. March 03, 2022, DOI:https://doi.org/10.1016/j.stemcr.2022.01.019.
  • Goettle, Schichel K, Reiche L, Werner L, Zink A, Prigione A, Küry P. TLR4 associated signaling disrupters as a new means to overcome HERV-W ENV mediated myelination deficits. Front Cell Neurosci. 2021 Nov 23;15:777542.
  • Prigione A. Stem Cell Research moving forward. Stem Cell Res, 2021 Aug 10;55:102503.
  • Palombo F, Peron C, Caporali L, Iannielli A, Maresca A, Di Meo I, Fiorini C, Segnali A, Sciacca F, Rizzo A, Levi S, Suomalainen A, Prigione A, Broccoli V, Carelli V, Tiranti V. The relevance of mitochondrial DNA variants fluctuation during reprogramming and neuronal differentiation of human iPSCs. Stem Cell Reports, July 29, 2021.
  • Brunetti D, Dykstra W, Le S, Zink A, and Prigione A. Mitochondria in neurogenesis: implications for mitochondrial diseases.  Stem Cells. 2021 Oct;39(10):1289-1297.
  • Inak G, Henke MT, and Prigione A. Bioenergetic profiling of human pluripotent stem cells.  Methods Mol Biol, 2021;2277:391-403.
  • Le S, Petersilie L, Inak G, Menacho-Pando C, Kafitz KW, Rybak-Wolf A, Rajewsky N, Rose CR, Prigione A. Generation of Human Brain Organoids for Mitochondrial Disease Modeling. JoVE, 2021 Jun 21;(172), doi: 10.3791/62756.
  • Inak G, Rybak-Wolf A, Lisowski P, Pentimalli TM, Jüttner R, Glažar P, Uppal K, Bottani E, Brunetti D, Secker C, Zink A, Meierhofer D, Henke MT, Dey M, Ciptasari U, Mlody B, Hahn T, Berruezo-Llacuna M, Karaiskos N, Di Virgilio M, Mayr JA, Wortmann SB, Priller J, Gotthardt M, Jones DP, Mayatepek E, Stenzel W, Diecke S, Kühn R, Wanker EE, Rajewsky N, Schuelke M, Prigione A. Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome.  Nat Commun. 2021, Mar 26;12(1):1929. DOI: 10.1038/s41467-021-22117-z. PMID: 33771987.
  • Zink, A., Lisowski, P. and Prigione, A. Generation of Human iPSC-derived Neural Progenitor Cells (NPCs) as Drug Discovery Model for Neurological and Mitochondrial Disorders. Bio-protocol 11(5): e3939. 2021; DOI: 10.21769/BioProtoc.3939.
  • Zink A, Conrad J, Telugu NS, Diecke S, Heinz A, Wanker EE, Priller J, and Prigione A. Assessment of ethanol-induced toxicity on iPSC-derived human neurons using a novel high-throughput mitochondrial neuronal health (MNH) assay. Front. Cell Dev. Biol. 2020 Oct 15; DOI: 10.3389/fcell.2020.590540.
  • Cunnane SC1, Trushina E, Morland C, Prigione A, Casadesus G, Andrews ZB, Beal MF, Bergersen LH, Brinton RD, de la Monte S, Eckert A, Harvey JZ, Jeggo R, Jhamandas JH, Kann O, Mannoury la Cour C, Martin WF, Mithieux G, Moreira PI, Murphy MP, Nave KA, Nuriel T, Oliet SHR, Saudou F, Mattson MP, Swerdlow RH, Millan MJ. Brain energy rescue: an emerging concept for new therapeutics in neurodegenerative disorders of aging. Nat Rev Drug Disc, 2020.
  • Delgado-Benito V, Berruezo-Llacuna M, Altwasser R, Winkler W, Sundaravinayagam D, Balasubramanian S, Caganova M, Graf R, Rahjouei A, Henke M, Driesner M, Keller L, Prigione A, Janz M, Akalin A, Di Virgilio M. Pdap1 protects mature B lymphocytes from stress-induced cell death and promotes antibody gene diversification. J Exp Med, 2020.
  • Laidou S, Alanis-Lobato G, Pribyl J, Rasko T, Tichy B, Mikulasek K, Tsagiopoulou M, Oppelt J, Kastrinaki G, Lefaki M, Singh M, Zink A, Chondrogianni N, Psomop F, Prigione A, Ivics Z, Popsilova S, Skadal P, Izsvak Z, Petrakis S. Nuclear inclusions of pathogenic ataxin-1 induce oxidative stress and perturb the protein synthesis machinery. Redox Biology, 11.02.2020, 101458.
  • Menacho C, Prigione A. Tackling mitochondrial diversity in brain function: from animal models to human brain organoids. Int J Biochem Cell Biol. 2020.
  • Lisowski P, Kannan P, Mlody B, Prigione A. Mitochondria and the dynamic control of stem cell homeostasis. EMBO Rep. 2018, May;19(5).
  • Zink A, Priller J, Prigione A. Pluripotent stem cells for uncovering the role of mitochondria in human brain function and dysfunction. J Mol Biol. 2018 Feb 16.
  • Scior A, Arnsburg K, Iburg M, Juenemann K, Pigazzini ML, Mlody B, Puchkov D, Ast A, Buntru A, Priller J, Wanker EE, Prigione A, Kirstein J. Complete suppression of Htt fibrilization and disaggregation of Htt fibrils by a trimeric chaperone complex. EMBO J. 2018 Jan 17;37(2):282-299.
  • Inak G, Lorenz C, Lisowski P, Zink A, Mlody B, Prigione A. iPSC-based drug discovery for mitochondrial disease. Stem Cells. 2017 Jul;35(7):1655-1662.
  • Lorenz C, Lesimple P, Bukowiecki R, Zink A, Inak G, Mlody B, Singh M, Semtner M, Mah N, Leong M, Auré K, Pfiffer V, Fauler B, Eichhorst J, Lyras EM, Wiesner B, Priller J, Huebner N, Mielke T, Meierhofer D, Izsvák Z, Meier JC, Bouillaud F, Adjaye J, Wanker E, Schuelke M, Lombès A, Prigione A. Human iPSC-derived neural progenitors are an effective drug discovery model for neurological mtDNA disorders. Cell Stem Cell. 2017, May 4;20(5):659-674.
  • Izsvák Z, Wang J, Singh M, Besser D, Prigione A, Ivics Z, Hurst L, Sun C. Isolation and Cultivation of Naïve-like Human Pluripotent Stem Cells based on LTR7/HERVH Expression. Nature Protocols. 2016, 11(2):327-346.
  • Wang J, Xie G, Singh M, Ghanbarian AT, Raskó T, Szvetnik A, Cai H, Besser D, Prigione A, Fuchs N, Schumann G, Chen W, Lorincz MC, Ivics Z, Hurst LD, Izsvák Z. Primate-specific endogenous retrovirus driven transcription defines naïve-like stem cells. Nature. 2014, Dec 18;516(7531):405-9.
  • Mlody B, Prigione A. A Glycolytic Solution for Pluripotent Stem Cells. Cell Stem Cell. 2016, Oct 6;19(4):419-420.
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