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Article

Generation of induced pluripotent stem cells from three individuals with Huntington's disease.
Miller, D.C., Lisowski, P., Lickfett, S., Mlody, B., Bünning, M., Genehr, C., Ulrich, C., Wanker, E.E., Diecke, S., Priller, J. and Prigione, A.
Stem Cell Research 65 : 102976. December 2022

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, M.T., Staege, S., Mlody, B., Bünning, M., Wanker, E., Diecke, S., Schuelke, M. and Prigione, A.
Stem Cell Research 61 : 102742. May 2022

Mitochondria in neurogenesis: implications for mitochondrial diseases.
Brunetti, D., Dykstra, W., Le, S., Zink, A. and Prigione, A.
Stem Cells 39 (10): 1289-1297. October 2021

Generation of human brain organoids for mitochondrial disease modeling.
Le, S., Petersilie, L., Inak, G., Menacho-Pando, C., Kafitz, K.W., Rybak-Wolf, A., Rajewsky, N., Rose, C.R. and Prigione, A.
Journal of Visualized Experiments (172): e62756. 21 June 2021

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, T.M., Jüttner, R., Glažar, P., Uppal, K., Bottani, E., Brunetti, D., Secker, C., Zink, A., Meierhofer, D., Henke, M.T., Dey, M., Ciptasari, U., Mlody, B., Hahn, T., Berruezo-Llacuna, M., Karaiskos, N., Di Virgilio, M., Mayr, J.A., Wortmann, S.B., Priller, J., Gotthardt, M., Jones, D.P., Mayatepek, E., Stenzel, W., Diecke, S., Kühn, R., Wanker, E.E., Rajewsky, N., Schuelke, M. and Prigione, A.
Nature Communications 12 (1): 1929. 26 March 2021

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 11 (5): e3939. 5 March 2021

Assessment of ethanol-induced toxicity on iPSC-derived human dopaminergic neurons using a novel high-throughput mitochondrial neuronal health (MNH) assay.
Zink, A., Conrad, J., Telugu, N.S., Diecke, S., Heinz, A., Wanker, E., Priller, J. and Prigione, A.
Frontiers in Cell and Developmental Biology 8 : 590540. 5 November 2020

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.T., Driesner, M., Keller, L., Prigione, A., Janz, M., Akalin, A. and Di Virgilio, M.
Journal of Experimental Medicine 217 (10): e20200137. 5 October 2020

Nuclear inclusions of pathogenic ataxin-1 induce oxidative stress and perturb the protein synthesis machinery.
Laidou, S., Alanis-Lobato, G., Pribyl, J., Raskó, T., Tichy, B., Mikulasek, K., Tsagiopoulou, M., Oppelt, J., Kastrinaki, G., Lefaki, M., Singh, M., Zink, A., Chondrogianni, N., Psomopoulos, F., Prigione, A., Ivics, Z., Pospisilova, S., Skladal, P., Izsvák, Z., Andrade-Navarro, M.A. and Petrakis, S.
Redox Biology 32 : 101458. May 2020

Novel calcineurin A (PPP3CA) variant associated with epilepsy, constitutive enzyme activation and downregulation of protein expression.
Rydzanicz, M., Wachowska, M., Cook, E.C., Lisowski, P., Kuźniewska, B., Szymańska, K., Diecke, S., Prigione, A., Szczałuba, K., Szybińska, A., Koppolu, A., Murcia Pienkowski, V., Kosińska, J., Wiweger, M., Kostrzewa, G., Brzozowska, M., Domańska-Pakieła, D., Jurkiewicz, E., Stawiński, P., Gromadka, A., Zielenkiewicz, P., Demkow, U., Dziembowska, M., Kuźnicki, J., Creamer, T.P. and Płoski, R.
European Journal of Human Genetics 27 (1): 61-69. January 2019

Complete suppression of Htt fibrilization and disaggregation of Htt fibrils by a trimeric chaperone complex.
Scior, A., Buntru, A., Arnsburg, K., Ast, A., Iburg, M., Juenemann, K., Pigazzini, M.L., Mlody, B., Puchkov, D., Priller, J., Wanker, E.E., Prigione, A. and Kirstein, J.
EMBO Journal 37 (2): 282-299. 17 January 2018

Human mesenchymal factors induce rat hippocampal- and human neural stem cell dependent oligodendrogenesis.
Jadasz, J.J., Tepe, L., Beyer, F., Samper Agrelo, I., Akkermann, R., Spitzhorn, L.S., Silva, M.E., Oreffo, R.O.C., Hartung, H.P., Prigione, A., Rivera, F.J., Adjaye, J. and Kuery, P.
Glia 66 (1): 145-160. January 2018

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., Auré, K., Leong, M., Zabiegalov, O., Lyras, E.M., Pfiffer, V., Fauler, B., Eichhorst, J., Wiesner, B., Huebner, N., Priller, J., Mielke, T., Meierhofer, D., Izsvák, Z., Meier, J.C., Bouillaud, F., Adjaye, J., Schuelke, M., Wanker, E.E., Lombès, A. and Prigione, A.
Cell Stem Cell 20 (5): 659-674.e9. 4 May 2017

A movement disorder with dystonia and ataxia caused by a mutation in the HIBCH gene.
Schottmann, G., Sarpong, A., Lorenz, C., Weinhold, N., Gill, E., Teschner, L., Ferdinandusse, S., Wanders, R.J.A., Prigione, A. and Schuelke, M.
Movement Disorders 31 (11): 1733-1739. November 2016

Isolation and cultivation of naive-like human pluripotent stem cells based on HERVH expression.
Wang, J., Singh, M., Sun, C., Besser, D., Prigione, A., Ivics, Z., Hurst, L.D. and Izsvák, Z.
Nature Protocols 11 (2): 327-346. February 2016

Annexin A1 sustains tumor metabolism and cellular proliferation upon stable loss of HIF1A.
Rohwer, N., Bindel, F., Grimm, C., Lin, S.J., Wappler, J., Klinger, B., Blüthgen, N., Du Bois, I., Schmeck, B., Lehrach, H., de Graauw, M., Goncalves, E., Saez-Rodriguez, J., Tan, P., Grabsch, H.I., Prigione, A., Kempa, S. and Cramer, T.
Oncotarget 7 (6): 6693-6710. 29 December 2015

Induced pluripotent stem cell-derived neuronal cells from a sporadic Alzheimer's disease donor as a model for investigating AD-associated gene regulatory networks.
Hossini, A.M., Megges, M., Prigione, A., Lichtner, B., Toliat, M.R., Wruck, W., Schroeter, F., Nuernberg, P., Kroll, H., Makrantonaki, E., Zoubouliss, C.C. and Adjaye, J.
BMC Genomics 16 (1): 84. 14 February 2015

Induced pluripotent stem cells (iPSCs) for modeling mitochondrial DNA disorders.
Prigione, A.
Methods in Molecular Biology 1265 : 349-356. 6 January 2015

Assessing the bioenergetic profile of human pluripotent stem cells.
Pfiffer, V. and Prigione, A.
Methods in Molecular Biology 1264 : 279-288. 2015

Primate-specific endogenous retrovirus-driven transcription defines naive-like stem cells.
Wang, J., Xie, G., Singh, M., Ghanbarian, A.T., Raskó, T., Szvetnik, A., Cai, H., Besser, D., Prigione, A., Fuchs, N.V., Schumann, G.G., Chen, W., Lorincz, M.C., Ivics, Z., Hurst, L.D. and Izsvák, Z.
Nature 516 (7531): 405-409. 18 December 2014

HIF1α modulates reprogramming through early glycolytic shift and up-regulation of PDK1-3 and PKM2.
Prigione, A., Rohwer, N., Hoffman, S., Mlody, B., Drews, K., Bukowiecki, R., Bluemlein, K., Wanker, E.E., Ralser, M., Cramer, T. and Adjaye, J.
Stem Cells 32 (2): 364-376. February 2014

Mitochondrial function in pluripotent stem cells and cellular reprogramming.
Bukowiecki, R., Adjaye, J. and Prigione, A.
Gerontology 60 (2): 174-182. February 2014

Teratoma formation of human embryonic stem cells in three-dimensional perfusion culture bioreactors.
Stachelscheid, H., Wulf-Goldenberg, A., Eckert, K., Jensen, J., Edsbagge, J., Bjoerquist, P., Rivero, M., Strehl, R., Jozefczuk, J., Prigione, A., Adjaye, J., Urbaniak, T., Bussmann, P., Zeilinger, K. and Gerlach, J.C.
Journal of Tissue Engineering and Regenerative Medicine 7 (9): 729-741. September 2013

Transcriptome based identification of mouse cumulus cell markers that predict the developmental competence of their enclosed antral oocytes.
Vigone, G., Merico, V., Prigione, A., Mulas, F., Sacchi, L., Gabetta, M., Bellazzi, R., Redi, C.A., Mazzini, G., Adjaye, J., Garagna, S. and Zuccotti, M.
BMC Genomics 14 : 380. 7 June 2013

Human stromal (mesenchymal) stem cells from bone marrow, adipose tissue and skin exhibit differences in molecular phenotype and differentiation potential.
Al-Nbaheen, M., Vishnubalaji, R., Ali, D., Bouslimi, A., Al-Jassir, F., Megges, M., Prigione, A., Adjaye, J., Kassem, M. and Aldahmash, A.
Stem Cell Reviews and Reports 9 (1): 32-43. February 2013

OCT4 and the acquisition of oocyte developmental competence during folliculogenesis.
Zuccotti, M., Merico, V., Belli, M., Mulas, F., Sacchi, L., Zupan, B., Redi, C.A., Prigione, A., Adjaye, J., Bellazzi, R. and Garagna, S.
International Journal of Developmental Biology 56 (10-12): 853-858. 2012

Mitochondrial-associated cell death mechanisms are reset to an embryonic-like state in aged donor-derived iPS cells harboring chromosomal aberrations.
Prigione, A., Hossini, A.M., Lichtner, B., Serin, A., Fauler, B., Megges, M., Lurz, R., Lehrach, H., Makrantonaki, E., Zouboulis, C.C and Adjaye, J.
PLoS ONE 6 (11): e27352. 14 November 2011

Human induced pluripotent stem cells harbor homoplasmic and heteroplasmic mitochondrial DNA mutations while maintaining human embryonic stem cell-like metabolic reprogramming.
Prigione, A., Lichtner, B., Kuhl, H., Struys, E.A., Wamelink, M., Lehrach, H., Ralser, M., Timmermann, B. and Adjaye, J.
Stem Cells 29 (9): 1338-1348. September 2011

Molecular insights into reprogramming-initiation events mediated by the OSKM gene regulatory network.
Mah, N., Wang, Y., Liao, M.C., Prigione, A., Jozefczuk, J., Lichtner, B., Wolfrum, K., Haltmeier, M., Floettmann, M., Schaefer, M., Hahn, A., Mrowka, R., Klipp, E., Andrade-Navarro, M.A. and Adjaye, J.
PLoS ONE 6 (8): e24351. 31 August 2011

Gatekeeper of pluripotency: a common Oct4 transcriptional network operates in mouse eggs and embryonic stem cells.
Zuccotti, M., Merico, V., Bellone, M., Mulas, F., Sacchi, L., Rebuzzini, P., Prigione, A., Redi, C.A., Bellazzi, R., Adjaye, J. and Garagna, S.
BMC Genomics 12 : 345. 5 July 2011

Comparative analysis of human embryonic stem cell and induced pluripotent stem cell-derived hepatocyte-like cells reveals current drawbacks and possible strategies for improved differentiation.
Jozefczuk, J., Prigione, A., Chavez, L. and Adjaye, J.
Stem Cells and Development 20 (7): 1259-1275. July 2011

A large-scale genetic association study to evaluate the contribution of Omi/HtrA2 (PARK13) to Parkinson's disease.
Krueger, R., Sharma, M., Riess, O., Gasser, T., Van Broeckhoven, C., Theuns, J., Aasly, J., Annesi, G., Bentivoglio, A.R., Brice, A., Djarmati, A., Elbaz, A., Farrer, M., Ferrarese, C., Gibson, J.M., Hadjigeorgiou, G.M., Hattori, N., Ioannidis, J.P.A., Jasinska-Myga, B., Klein, C., Lambert, J.C., Lesage, S., Lin, J.J., Lynch, T., Mellick, G.D., de Nigris, F., Opala, G., Prigione, A., Quattrone, A., Ross, O.A., Satake, W., Silburn, P.A., Tan, E.K., Toda, T., Tomiyama, H., Wirdefeldt, K., Wszolek, Z., Xiromerisiou, G. and Maraganore, D.M.
Neurobiology of Aging 32 (3): 548.e9-548.e18. March 2011

An ES-like pluripotent state in FGF-dependent murine iPS cells.
Di Stefano, B., Buecker, C., Ungaro, F., Prigione, A., Chen, H.H., Welling, M., Eijpe, M., Mostoslavsky, G., Tesar, P., Adjaye, J., Geijsen, N. and Broccoli, V.
PLoS ONE 5 (12): e16092. 30 December 2010

The LARGE principle of cellular reprogramming: lost, acquired and retained gene expression in foreskin and amniotic fluid-derived human iPS cells.
Wolfrum, K., Wang, Y., Prigione, A., Sperling, K., Lehrach, H. and Adjaye, J.
PLoS ONE 5 (10): e13703. 29 October 2010

Alpha-synuclein nitration and autophagy response are induced in peripheral blood cells from patients with Parkinson disease.
Prigione, A., Piazza, F., Brighina, L., Begni, B., Galbussera, A., DiFrancesco, J.C., Andreoni, S., Piolti, R. and Ferrarese, C.
Neuroscience Letters 477 (1): 6-10. 14 June 2010

A transcriptional roadmap to the induction of pluripotency in somatic cells.
Wang, Y., Mah, N., Prigione, A., Wolfrum, K., Andrade-Navarro, M.A. and Adjaye, J.
Stem Cell Reviews 6 (2): 282-296. June 2010

Lymphomonocyte alpha-synuclein levels in aging and in Parkinson disease.
Brighina, L., Prigione, A., Begni, B., Galbussera, A., Andreoni, S., Piolti, R. and Ferrarese, C.
Neurobiology of Aging 31 (5): 884-885. May 2010

The senescence-related mitochondrial/oxidative stress pathway is repressed in human induced pluripotent stem cells.
Prigione, A., Fauler, B., Lurz, R., Lehrach, H. and Adjaye, J.
Stem Cells 28 (4): 721-733. April 2010

Modulation of mitochondrial biogenesis and bioenergetic metabolism upon in vitro and in vivo differentiation of human ES and iPS cells.
Prigione, A. and Adjaye, J.
International Journal of Developmental Biology 54 (11-12): 1729-1741. 2010

Efficient genetic reprogramming of unmodified somatic neural progenitors uncovers the essential requirement of Oct4 and Klf4.
Di Stefano, B., Prigione, A. and Broccoli, V.
Stem Cells and Development 18 (5): 707-716. June 2009

Mitochondrial DNA deletions induce the adenosine monophosphate-activated protein kinase energy stress pathway and result in decreased secretion of some proteins.
Prigione, A. and Cortopassi, G.
Aging Cell 6 (5): 619-630. October 2007

Mitochondrial DNA deletions and chloramphenicol treatment stimulate the autophagic transcript ATG12.
Prigione, A. and Cortopassi, G.
Autophagy 3 (4): 377-380. July 2007

Mitochondrial DNA deletions inhibit proteasomal activity and stimulate an autophagic transcript.
Alemi, M., Prigione, A., Wong, A., Schoenfeld, R., DiMauro, S., Hirano, M., Taroni, F. and Cortopassi, G.
Free Radical Biology and Medicine 42 (1): 32-43. 1 January 2007

Oxidative stress in peripheral blood mononuclear cells from patients with Parkinson's disease: negative correlation with levodopa dosage.
Prigione, A., Begni, B., Galbussera, A., Beretta, S., Brighina, L., Garofalo, R., Andreoni, S., Piolti, R. and Ferrarese, C.
Neurobiology of Disease 23 (1): 36-43. July 2006

"What do you remember about Chernobyl?" A new test of memory for media-mediated events.
Bizzozero, I., Lucchelli, F., Prigione, A., Saetti, M.C. and Spinnler, H.
Neurological Sciences 25 (4): 205-215. October 2004

Review

Tackling mitochondrial diversity in brain function: from animal models to human brain organoids.
Menacho, C. and Prigione, A.
International Journal of Biochemistry and Cell Biology 123 : 105760. June 2020

Live-imaging readouts and cell models for phenotypic profiling of mitochondrial function.
Iannetti, E.F., Prigione, A., Smeitink, J.A.M., Koopman, W.J.H., Beyrath, J. and Renkema, H.
Frontiers in Genetics 10 : 131. March 2019

Mitochondria and the dynamic control of stem cell homeostasis.
Lisowski, P., Kannan, P., Mlody, B. and Prigione, A.
EMBO Reports 19 (5): e45432. 1 May 2018

Pluripotent stem cells for uncovering the role of mitochondria in human brain function and dysfunction.
Zink, A., Priller, J. and Prigione, A.
Journal of Molecular Biology 430 (7): 891-903. 30 March 2018

Mitochondrial metabolism in early neural fate and its relevance for neuronal disease modeling.
Lorenz, C. and Prigione, A.
Current Opinion in Cell Biology 49 : 71-76. December 2017

Concise review: Induced pluripotent stem cell-based drug discovery for mitochondrial disease.
Inak, G., Lorenz, C., Lisowski, P., Zink, A., Mlody, B. and Prigione, A.
Stem Cells 35 (7): 1655-1662. July 2017

The return of metabolism: biochemistry and physiology of the pentose phosphate pathway.
Stincone, A., Prigione, A., Cramer, T., Wamelink, M.M.C., Campbell, K., Cheung, E., Olin-Sandoval, V., Gruening, N.M., Krueger, A., Tauqeer Alam, M., Keller, M.A., Breitenbach, M., Brindle, K.M., Rabinowitz, J.D. and Ralser, M.
Biological Reviews 90 (3): 927-963. August 2015

Metabolic restructuring and cell fate conversion.
Prigione, A., Ruiz-Perez, M.V., Bukowiecki, R. and Adjaye, J.
Cellular and Molecular Life Sciences 72 (9): 1759-1777. 1 May 2015

Human induced pluripotent stem cells--from mechanisms to clinical applications.
Drews, K., Jozefczuk, J., Prigione, A. and Adjaye, J.
Journal of Molecular Medicine 90 (7): 735-745. July 2012

Letter

Increased oxidative stress in lymphocytes from untreated Parkinson's disease patients.
Prigione, A., Isaias, I.U., Galbussera, A., Brighina, L., Begni, B., Andreoni, S., Pezzoli, G., Antonini, A. and Ferrarese, C.
Parkinsonism & Related Disorders 15 (4): 327-328. May 2009

Editorial

A glycolytic solution for pluripotent stem cells.
Mlody, B. and Prigione, A.
Cell Stem Cell 19 (4): 419-420. 6 October 2016

A mitochondrial view of cell fate.
Prigione, A.
Seminars in Cell & Developmental Biology 52 : 66-67. April 2016

Comment

Aging vs. rejuvenation: reprogramming to iPSCs does not turn back the clock for somatic mitochondrial DNA mutations.
Lorenz, C. and Prigione, A.
Stem Cell Investigation 3 : 43. September 2016

A mitochondrial strategy for safeguarding the reprogrammed genome.
Prigione, A. and Adjaye, J.
Cell Regeneration 3 (1): 5. 29 March 2014