Publikationen ǀ UKD

Source: Human iPSC-derived cortical organoid stained with SOX2 (red) and MAP2 (Cyan). image by Stephanie Le

Selected publications

Retrospective natural history of mitochondrial ATPase deficiency of in a large cohort of unreported patients.
Carli S, Levaret A, Diodato D, Bertini E, Martinelli D, Malandrini A, Lopergolo D, Gallus GN, Ganetsky R, La Morgia C, Carelli V, Primiano G, Dominguez C, Lorenzo PS, Martin MA, Ardissone A, Lamperti C, Nicoletta V, Klopstock T, Distelmaier F, Zeng L, Buechner B, Mancuso M, Schuelke M, Prigione A, Garone C.
Neurology. 2025 April: link
Patients with Allan-Herndon-Dudley Syndrome (MCT8 Deficiency) Display Symptoms of Parkinsonism in Childhood and Respond to Levodopa/Carbidopa Treatment.
Wilpert N-M, Hewitt AL. Pons R, Henke M-T, Dell'Orco A, Bauer M, Grolik C, Menz S, Wahle M, Zink A, Prigione A, Reinauer C, Lange C, Furth C, Brockmann K, Jung-Klawitter S, Christ S, Kaindl AM, Tietze A, Krude H, Opladen T, Schuelke M.
Movement Disorders. March 2025 : link
SIMPATHIC: Accelerating drug repurposing for rare diseases by exploiting SIMilarities in clinical and molecular PATHology
van Karnebeek CDM, Müller AR, Benkemoun L, Boussaad I, Cornel MC, IntHout J, de Kort M, de Oliveira Martins S, Prigione A, Rigter T, Roes KCB, Sanchez A, Schipper R, Wilkinson MD, ’t Hoen PAC
Mol Genet Metab. 2025 March: link
iPSC models of mitochondrial diseases
Heiduschka S, Prigione A
Neurobiol Dis. 2025 Jan: link
Disease models of Leigh syndrome: From yeast to organoids
Henke MT, Prigione A, Schuelke M
J Inherit Metab Dis. 2024 Dec: link
Reassessment of marker genes in human induced pluripotent stem cells for enhanced quality control
Dobner J, Diecke S, Krutmann J, Prigione A, Rossi A
Nature Communication. 2024 Oct: link
Fetal and obstetrics manifestations of mitochondrial diseases
Adelizzi A, Giri A, Di Donfrancesco A, Boito S, Prigione A, Bottani E, Bollati V, Tiranti V, Persico N, Brunetti D.
J Transl Med. 2024 Sept: link
Mutant Huntingtin impairs neurodevelopment in human brain organoids through CHCHD2-mediated neurometabolic failure
Lisowski P, Lickfett S, Rybak-Wolf A, Menacho C, Le S, Pentimalli TM, Notopoulou S, Dykstra W, Oehler D, Lopez-Calcerrada S, Mlody B, Otto M, Wu H, Richter Y, Roth P, Anand R, Kulka LAM, Meierhofer D, Glazar P, Legnini I, Telugu NS, Hahn T, Neuendorf N, Miller DC, Böddrich A, Polzin A, Mayatepek E, Diecke S, Olzscha H, Kirstein J, Ugalde C, Petrakis S, Cambridge S, Rajewsky N, Kühn R, Wanker EE, Priller J, Metzger JJ*, and Prigione A*
Nature Communication. 2024 Aug: link
*co-last authors
Protocol for the generation of cultured cortical brain organoid slices (cBOS)
Petersilie L, Kafitz KW, Neu L, Heiduschka S, Le S, Prigione A*, Rose CR*
STAR Protocols. 2024 Aug: link
*co-last authors
Balancing neuronal activity to fight neurodevelopmental disorders
Le S, Menacho C, Prigione A
Trends in Neuroscience. 2024 Mar: link
mtDNA Analysis using Mitopore
Dobner JTN, Nguyen T, Pavez-Giani MG, Cyganek L, Distelmaier F, Krutmann J, Prigione A, Rossi A
Mol Ther Methods Clin Dev. 2024 Mar: link
Cortical brain organoid slices (cBOS) as a functional model system to study human neural cells in intact minimal networks
Petersilie L, Heiduschka S, Nelson JSE, Neu LA, Le S, Anand R, Kafitz KW, Prigione A*, Rose CR*
iScience. 2024 Mar: link
*co-last authors
Deep learning-driven neuromorphogenesis screenings identify repurposable drugs for mitochondrial disease
Menacho C, Okawa S, Álvarez-Merz I, Wittich A, Muñoz-Oreja M, Lisowski P, Pentimalli TM, Rybak-Wolf A, Inak G, Zakin S, Thevandavakkam M, Petersilie L, Zaliani A, Mlody B, Seibt A, Donnelly J, Woleben K, Fernandez-Checa J, Herebian D, Mayatepek E, Rajewsky N, Spinazzola A, Schuelke M, Perlstein E, Rossi A, Distelmaier F, Holt IJ, Pless O, Rose CR, Del Sol A*, and Prigione A*
BioRxiv. 2024: link
*co-last authors
Modeling mitochondrial DNA diseases: from base editing to pluripotent stem-cell-derived organoids
Tolle I, Tiranti V, Prigione A
EMBO Reports. 2023 Mar: link
Activated microglia and neuroinflammation as a pathogenic mechanism in Leigh syndrome
Daneshgar N, Leidinger MR, Le S, Hefti M, Prigione A, Dai DF
Frontiers Neuroscience. 2023 Jan: link
A Novel Gene Controls a New Structure: PiggyBac Transposable Element-Derived 1, Unique to Mammals, Controls Mammal-Specific Neuronal Paraspeckles
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
Mol Biol Evol. 2022 Oct: link
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
Steiner T, Zink A, Henke MT, Cecchetto G, Buenning M, Rossi A, Schuelke M, Prigione A
Stem Cell Res. 2022 Sept: link
High-content analysis of neuronal morphology in human iPSC-derived neurons
Lickfett S, Menacho C, Zink A, Telugu NS, Beller M, Diecke S, Cambridge S, Prigione A
STAR Protocol 2022 Aug: link
High-content screening of mitochondrial polarization in neural cells derived from human pluripotent stem cells
Zink A, Haferkamp U, Wittich A, Beller M, Pless O, Prigione A
STAR Protocol. 2022 Aug: link
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
Lorenz C, Zink A, Henke MT, Staege S, Mlody B, Bünning M, Wanker E, Diecke S, Schuelke M, Prigione A.
Stem Cell Res. 2022: link
A call for consensus guidelines on monitoring the integrity of nuclear and mitochondrial genome in human pluripotent stem cells
Rossi A, Lickfett S, Martins S, Prigione A
Stem Cell Reports. 2022 Mar: link
TLR4 associated signaling disrupters as a new means to overcome HERV-W ENV mediated myelination deficits
Goettle P, Schichel K, Reiche L, Werner L, Zink A, Prigione A, Küry P
Front Cell Neuroscience. 2021 Nov: link
Mitochondria in neurogenesis: implications for mitochondrial diseases
Brunetti D, Dykstra W, Le S, Zink A, and Prigione A
Stem Cells. 2021 Oct: link
Stem Cell Research moving forward
Prigione A
Stem Cell Res. 2021 Aug: link
The relevance of mitochondrial DNA variants fluctuation during reprogramming and neuronal differentiation of human iPSCs
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
Stem Cell Reports. 2021 July: link
Bioenergetic profiling of human pluripotent stem cells
Inak G, Henke MT, and Prigione A
Methods Mol Biol. 2021 Jun: link
Generation of Human Brain Organoids for Mitochondrial Disease Modeling
Le S, Petersilie L, Inak G, Menacho-Pando C, Kafitz KW, Rybak-Wolf A, Rajewsky N, Rose CR, Prigione A
JoVE. 2021 Jun: link
Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome
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
Nature Communication. 2021 Mar: link
Generation of Human iPSC-derived Neural Progenitor Cells (NPCs) as Drug Discovery Model for Neurological and Mitochondrial Disorders
Zink, A., Lisowski, P. and Prigione, A
Bio-protocol. 2021 Mar: link
Assessment of ethanol-induced toxicity on iPSC-derived human neurons using a novel high-throughput mitochondrial neuronal health (MNH) assay
Zink A, Conrad J, Telugu NS, Diecke S, Heinz A, Wanker EE, Priller J, and Prigione A
Front. Cell Dev. Biol. 2020 Oct: link
Brain energy rescue: an emerging concept for new therapeutics in neurodegenerative disorders of aging
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
Nat Rev Drug Disc. 2020 Sept: link
PDGFA-associated protein 1 protects mature B lymphocytes from stress-induced cell death and promotes antibody gene diversification
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
J Exp Med. 2020 Oct: link
Nuclear inclusions of pathogenic ataxin-1 induce oxidative stress and perturb the protein synthesis machinery
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
Redox Biology. 2020 May: link
Tackling mitochondrial diversity in brain function: from animal models to human brain organoids
Menacho C, Prigione A
Int J Biochem Cell Biol. 2020: link
Mitochondria and the dynamic control of stem cell homeostasis
Lisowski P, Kannan P, Mlody B, Prigione A
EMBO Reports. 2018 Apr: link
Pluripotent stem cells for uncovering the role of mitochondria in human brain function and dysfunction
Zink A, Priller J, Prigione A
J Mol Biol. 2018 Feb: link
Complete suppression of Htt fibrilization and disaggregation of Htt fibrils by a trimeric chaperone complex
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
EMBO J. 2018 Jan: link
A Glycolytic Solution for Pluripotent Stem Cells
Mlody B, Prigione A
Cell Stem Cell. 2016 Oct: link
iPSC-based drug discovery for mitochondrial disease
Inak G, Lorenz C, Lisowski P, Zink A, Mlody B, Prigione A
Stem Cells. 2017 Jul: link
Human iPSC-derived neural progenitors are an effective drug discovery model for neurological mtDNA disorders
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
Cell Stem Cell. 2017 May: link
Isolation and Cultivation of Naïve-like Human Pluripotent Stem Cells based on LTR7/HERVH Expression
Wang J, Singh M, Sun C, Besser D, Prigione A, Ivics Z, Hurst L, Izsvák Z.
Nature Protocols. 2016 Jan: link
Primate-specific endogenous retrovirus driven transcription defines naïve-like stem cells
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
Nature. 2014 Oct: link

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Selected publications