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Associated Researchers

Associated Researchers and Clinician Scientists

2003 Diploma in Biology, University of Münster

2007 PhD, Institute of Genetics, Johannes-Gutenberg University of Mainz

2008 Postdoctoral Fellow, Institute of Stem Cell Research, Helmholtz Centre Munich

2012 Postdoctoral Fellow, Institute of Developmental Genetics, Helmholtz Centre Munich

2013 Senior Research Fellow, Institute of Biochemistry, FAU Erlangen-Nürnberg

2017 Independant Group Leader, Institute of Biochemistry, FAU Erlangen-Nürnberg

Research focus

Interaction of stem cells and their progeny with their microenvironment is critical for regulation of hippocampal neurogenesis. Astrocytes are considered major components of the hippocampal neurogenic niche, but their specific contribution to neurogenesis-dependent plasticity remains largely understudied. In contrast to the prevailing assumption that mature astrocytes are postmitotic in the non-injured adult brain, I recently identified astrocyte subtypes, which started to proliferate in response to pro- or anti-neurogenic stimuli, thus revealing unexpected astrocyte plasticity. In my research I pursue the novel hypothesis that astrocyte heterogeneity and astrogenesis are critical modulators of neurogenesis-dependent plasticity.

Publications

Schäffner I, Minakaki G, Khan MA, Balta EA, Schlötzer-Schrehardt U, Schwarz TJ, Beckervordersandforth R, Winner B, Webb AE, DePinho RA, Paik J, Wurst W, Klucken J, Lie DC (2018) FoxO Function Is Essential for Maintenance of Autophagic Flux and Neuronal Morphogenesis in Adult Neurogenesis. Neuron 99(6):1188-1203.e6. doi: 10.1016/j.neuron.2018.08.017

Fiebig C, Keiner S, Ebert B, Schäffner I, Jagasia R, Lie DC, Beckervordersandforth R (2019) Mitochondrial Dysfunction in Astrocytes Impairs the Generation of Reactive Astrocytes and Enhances Neuronal Cell Death in the Cortex Upon Photothrombotic Lesion. Frontiers in Molecular Neuroscience 12:40. doi: 10.3389/fnmol.2019.00040

Schäffner I, Minakaki G, Khan MA, Balta EA, Schlötzer-Schrehardt U, Schwarz T, Beckervordersandforth R, Winner B, Webb AE, DePinho R, Paik J, Wurst W, Klucken J, and Lie DC (2018) FoxO function is essential for maintenance of autophagic flux and neuronal morphogenesis in adult neurogenesis. Neuron 2018 Sep 19;99(6):1188-1203 (JIF: 13.9)

Pons-Espinal M, de Luca E, Marzi MJ, Beckervordersandforth R, Armirotti A, Nicassio F, Fabel K, Kempermann G, De Pietri Tonelli D (2017) Synergistic functions of miRNAs determine neuronal fate of adult neural stem cells. Stem Cell Reports, 2017 April 11; 8(4):1046-1061 (JIF: 8.2)

Beckervordersandforth R#*, Ebert B*, Schäffner I, Moss J, Fiebig C, Shin J, Moore DL, Ghosh L, Trinchero MF, Stockburger C, Friedland K, Steib K, von Wittgenstein J, Keiner S, Redecker C, Holter SM, Xiang W, Wurst W, Jagasia R, Schinder AF, Ming GL, Toni N, Jessberger S, Song HJ, Lie DC# (2017) Role of mitochondrial metabolism in the control of early lineage progression and aging phenotypes in adult hippocampal neurogenesis. Neuron 2017 Feb 8 93: 1-14 (JIF: 13.9) # Corresponding authors, * Equal contribution

Gebara E, Bonaguidi MA*, Beckervordersandforth R*, Sultan S, Udry F, Gijs PJ, Lie DC, Ming G, Song S, and Toni N. (2015) Heterogeneity of radial glia-like cells in the adult hippocampus. Stem Cells 2016 Jan 1; (JIF: 5.9) * Equal contribution

Fukusumi Y, Meier M, Götz S, Matheus F, Irmler M, Beckervordersandforth R, Faus-Kessler T, Minina E, Rauser B, Zhang J, Arenas E, Andersson E, Niehrs C, Beckers J,Simeone A, Wurst W, and Prakash N. (2015) Dickkopf 3 promotes the differentiation of a rostrolateral midbrain dopaminergic neural subset in vivo and from pluripotent stem cells in vitro in mouse. J Neurosci. 2015 September 30;35(39):13385-13401 (JIF: 6.3)

Karow M, Schichor C, Beckervordersandforth R, and Berninger B. (2014) Lineage reprogramming of pericyte-derived cells of the adult human brain into induced neurons. Journal of visualized experiments JoVE 01/2014 DOI:10.3791/51433 (JIF: 1.1)

Beckervordersandforth R#, Deshpande A, Schäffner I, Huttner HB, Lepier A, Lie CD, and Götz, M. (2014) In vivo targeting of adult neural stem cells in the dentate gyrus by a split-Cre approach Stem Cell Reports, 2014 Feb 6; 2(2);153-162 (JIF 8.2) # Corresponding author

Fischer J*, Beckervordersandforth R*, Tripathi P, Steiner-Mezzadri A, Ninkovic J, and Götz, M. (2011) Prospective isolation of the adult neural stem cells and their progeny from the mouse subependymal zone. Nat Protoc. 2011 Nov 17 ;6(12) :1981-9 (JIF: 9.6) * Equal contribution

Costa MR, Ortega F, Brill MS, Beckervordersandforth R, Petrone C, Schroeder T, Götz M,and Berninger B. (2012) Continuous live imaging of adult neural stem cell division and lineage progression in vitro. Development, 138 (2011), 1057-1068 (2011) (JIF: 6.1)

Eilken H, Rieger MA, Hoppe PS, Hermann AC, Smejkal BM, Drew EC, Thum M, Ninkovic J, Beckervordersandforth R, and Schroeder T. (2011) Continuous long-term detection of live cell surface markers by ‘in culture’ antibody staining. Nature Protocol Exchange 2011 Jan (28); doi:10.1038/protex.2011.205

Beckervordersandforth R*, Tripathi P*, Ninkovic J*, Bayam E, Lepier A, Stempfhuber B,Kirchhoff F, Hirrlinger J, Haslinger A, Lie DC, Beckers J, Yoder B, Irmler M, and Götz M.(2010) In vivo fate mapping and expression analysis reveals molecular hallmarks of prospectively isolated adult neural stem cells. Cell Stem Cell. 2010 Dec 3;7(6):744-58 (JIF:22.4) * Equal contribution

Gajera CR, Emich H, Lioubinski O, Christ A, Beckervordersandforth-Bonk R, Yoshikawa K, Bachmann S, Christensen EI, Götz M, Kempermann G, Perterson AS, Willnow TE, and Hammes A. (2010) LRP2 in ependymal cells regulates BMP signalling in the adult neurogenic niche. J Cell Sci. 2010 Jun 1;123(Pt11):1922-30. Epub 2010 May11 (JIF: 4.7)

Beckervordersandforth RM, Rickert C, Altenhein B, and Technau GM. (2008) Subtypes of glial cells in the Drosophila embryonic ventral nerve cord as related to lineage and gene expression. Mech Dev. 2008 May-Jun;125(5-6):542-57. Epub 2008 Jan 8 (JIF: 1.9)

von Hilchen CM, Beckervordersandforth RM, Rickert C, Technau GM, and Altenhein B. (2008) Identity, origin, and migration of peripheral glial cells in the Drosophila embryo. Mech Dev. 2008 Mar-Apr;125(3-4):337-52. Epub 2007 Nov 1 (JIF: 1.9)

BOOK CHAPTER

Schneider J, Karpf J, Beckervordersandforth R (2019) Role of Astrocytes in the neurogenic niches. Methods in Molecular Biology, vol. 1938

Beckervordersandforth R, Zhang CL, and Lie DC. 2015 Transcrition factor-dependent control of adult hippocampal neurogenesis Cold Spring Harb Perspecti. Biol doi: 10.1101/cshperspect.a018879 (JIF: 8.7)

REVIEW/PREVIEW

Beckervordersandforth R, Rolando C (2019) Untangling human neurogenesis to understand and counteract brain disorders. Curr Opin Pharmacol 50: 67-73. doi: 10.1016/j.coph.2019.12.002

Beckervordersandforth R (2017) Newborn neurons in the aged brain – scarce and slow but highly plastic. Cell Reports 21, October 31, 2017

Beckervordersandforth R (2017) Mitochondrial metabolism-mediated regulation of adult neurogenesis. Brain Plasticity 3 (2017) 73-87

Beckervordersandforth R, Häberle BM, and Lie DC. (2015) Metabolic regulation of adult stem cell-derived neurons. Front.Biol., 2015, 10(2):107-116

Dr. Melanie Küspert

Group Leader

Institute of Biochemistry
Chair of Biochemistry and Pathobiochemistry

2008 Diploma degree in Biology, FAU Erlangen-Nürnberg

2008 Graduate student, FAU Erlangen-Nürnberg

2012 Postdoctoral fellow, Institute of Biochemistry, FAU Erlangen-Nürnberg

2019 Group leader, Institute of Biochemistry, FAU Erlangen-Nürnberg

Research focus

My research focuses on the identification and functional analysis of novel regulators and modifiers of glial development and myelination in vitro and in vivo. To obtain a better understanding of transcriptional regulatory networks, which jointly work together during glial development to enable proper myelination and remyelination, I focus on potential targets and interactors of known key regulators of myelination processes, such as the transcription factors Sox10 and Myrf in oligodendrocytes.

Publications

Wittstatt J, Reiprich S, Küspert M.: Crazy Little Thing Called Sox-New Insights in Oligodendroglial Sox Protein Function. Int J Mol Sci. 2019 Jun 2;20(11). pii: E2713. doi: 10.3390/ijms20112713. Review.

Elsesser O, Fröb F, Küspert M, Tamm ER, Fujii T, Fukunaga R, Wegner M.: Chromatin remodeler Ep400 ensures oligodendrocyte survival and is required for myelination in the vertebrate central nervous system. Nucleic Acids Res. 2019 Jul 9;47(12):6208-6224. doi: 10.1093/nar/gkz376.

Cantone M* & Küspert M* & Reiprich S*, Lai X, Eberhardt M, Göttle P, Beyer F, Azim K, Küry P, Wegner M & Vera J (2018) A gene regulatory architecture that controls regionindependent dynamics of oligodendrocyte differentiation. Glia. Accepted for publication. (*authors contributed equally to this publication)

Jacob A* & Wüst HM*, Thalhammer JM, Fröb F, Küspert M, Reiprich S, Balta E-A, Lie DC, Wegner M, Sock E (2018) The homeodomain transcription factor Prox1 is a direct target of SoxC proteins during developmental vertebrate neurogenesis. Journal of Neurochemistry 146(3):251-268. *equal contribution

Weider M* & Starost LJ* & Groll K*, Küspert M, Sock E, Wedel M, Fröb F, Schmitt C, Baroti T, Hartwig AC, Hillgärtner S, Piefke S, Fadler T, Ehrlich M, Ehlert C, Stehling M, Albrecht S, Jabali A, Schöler HR, Winkler J, Kuhlmann T, Wegner M. (2018) Nfat/calcineurin signaling promotes oligodendrocyte differentiation and myelination by transcription factor network tuning. Nature Communications 9(1):899. *equal contribution

Reiprich S* & Cantone M* & Weider M*, Baroti T, Wittstatt J, Schmitt C, Küspert M, Vera J, Wegner M (2017) Transcription factor Sox10 regulates oligodendroglial Sox9 levels via microRNAs. Glia 65(7):1089-1102. *equal contribution

Wegener A, Küspert M, Sock E, Philipsen S, Suske G, Wegner M (2017) Sp2 is the only glutamine-rich specificity protein with minor impact on development and differentiation in myelinating glia. Journal of Neurochemistry 140(2):245-256.

Mehta P, Küspert M, Bale T, Brownstein CA, Towne MC, De Girolami U, Shi J, Beggs AH, Darras BT, Wegner M, Piao X, Agrawal PB (2017) Novel Mutation in CNTNAP1 results in Congenital Hypomyelinating Neuropathy. Muscle & Nerve 55(5):761-765.

Muth KN, Piefke S, Weider M, Sock E, Hermans-Borgmeyer I, Wegner M, Küspert M (2016) The Dual-specificity phosphatase Dusp15 is regulated by Sox10 and Myrf in Myelinating Oligodendrocytes. Glia 64(12):2120-2132.

Küspert M, Wegner M (2016) SomethiNG 2 talk about-Transcriptional regulation in embryonic and adult oligodendrocyte precursors. Brain Research 1638(Pt B):167-182. Review

Weider M & Wegener A, Schmitt C, Küspert M, Hillgärtner S, Bösl MR, Hermans-Borgmeyer I, Nait-Oumesmar B, Wegner M (2015) Elevated In Vivo Levels of a Single Transcription Factor Directly Convert Satellite Glia into Oligodendrocyte-like Cells. PLoS Genetics 11(2) e1005008.

Bischof M, Weider M, Küspert M, Nave KA, Wegner M (2015) Brg1-dependent Chromatin remodelling is not essentially required during oligodendroglial differentiation. Journal of Neuroscience 35(1):21-35.

Hoffmann S, Hos D, Küspert M, Lang RA, Lovell-Badge R, Wegner M, Reiprich S (2014) Stem cell factor Sox2 and its close relative Sox3 have differentiation functions in oligodendrocytes. Development 141(1):39-50.

Vogl MR, Reiprich S, Küspert M, Kosian T, Schrewe H, Nave KA, Wegner M (2013) Sox10 Cooperates with the Mediator Subunit 12 during Terminal Differentiation of Myelinating Glia. Journal of Neuroscience 33(15):6679-6690.

Weider M* & Küspert M*, Bischof M, Vogl MR, Hornig J, Loy K, Kosian T, Müller J, Hillgärtner S, Tamm ER, Metzger D, Wegner M (2012) Chromatin-Remodeling Factor Brg1 Is Required for Schwann Cell Differentiation and Myelination. Developmental Cell 23(1):193-201.(*authors contributed equally to this publication)

Küspert M, Weider M, Müller J, Hermans-Borgmeyer I, Meijer D, Wegner M (2012) Desert hedgehog links transcription factor Sox10 to perineurial development. Journal of Neuroscience 32(16):5472-5480.

Küspert M, Hammer A, Bösl MR, Wegner M (2011) Olig2 regulates Sox10 expression in oligodendrocyte precursors through an evolutionary conserved distal enhancer. Nucleic Acids Research 39(4):1280-1293.

Walter B, Küspert M, Ansorge D, Krämer R, Burkovski A (2008) Dissection of Ammonium uptake systems in Corynebacterium glutamicum: mechanism of action and energetics ofAmtA and AmtB. Journal of Bacteriology 190(7):2611-2614.

 

Dr. Christiane Mühle

Group Leader

Department of Psychiatry and Psychotherapy
Chair of Psychiatry and Psychotherapy

2001 Diploma in Biochemistry, Leipzig University

2001 PhD student at the Friedrich-Alexander University of Erlangen-Nuernberg (FAU)

2007 Research Assistant at the Medical University of Innsbruck (MUI)

2008 Postdoctoral Fellow at the Department of Psychiatry and Psychotherapy, University Hospital Erlangen/ FAU

2013 Head of departmental biobanking at the Molecular Neurobiology Laboratories

2019 Group Leader “Biomarkers in Psychiatry”, Department of Psychiatry and Psychotherapy, University Hospital Erlangen/ FAU

Research focus

Recent data strongly indicate the influence of the sphingolipid metabolism in the pathophysiology of neuropsychiatric diseases such as depression, alcohol addiction and neurodegeneration. We characterize the involved sphingomyelinase and ceramidase enzymes in various biological materials and investigate their genetic variation, activity and regulation in animal / cell culture models, healthy individuals and patients. Thereby we aim to better understand the underlying molecular mechanisms as well as to evaluate the suitability of these enzymes as diagnostic or prognostic biomarkers and as preventive and therapeutic targets.

 

Publications

Lenz B, Mühle C, Cohort Study on Substance Use Risk F, Kornhuber J: Lower digit ratio (2D:4D) in alcohol dependence: Confirmation and exploratory analysis in a population-based study of young men. Addict Biol 2019: e12815.

Buchholz VN, Mühle C, C-SURF, Kornhuber J, Lenz B: Lower Digit Ratio (2D:4D) Indicative of Excess Prenatal Androgen Is Associated With Increased Sociability and Greater Social Capital. Front. Behav. Neurosci. 2019;13:246.

Lenz B, Köllner MG, Mühle C, Weinland C, Kornhuber J (2019a) Basic Human Body Dimensions Relate to Alcohol Dependence and Predict Hospital Readmission. J Clin Med 8:12.

Kalinichenko LS, Mühle C, Eulenburg V, Praetner M, Reichel M, Gulbins E, Kornhuber J, Muller CP (2019) Enhanced Alcohol Preference and Anxiolytic Alcohol Effects in Niemann-Pick Disease Model in Mice. Front Neurol 10: 731.

Mühle C, Wagner CJ, Färber K, Richter-Schmidinger T, Gulbins E, Lenz B, Kornhuber J: Secretory acid sphingomyelinase in the serum of medicated patients predicts the prospective course of depression. Journal of Clinical Medicine 2019;8.

Weinland C, Mühle C, Kornhuber J, Lenz B: Crossed eye/hand laterality and left-eyedness predict a positive 24-month outcome in alcohol-dependent patients. Alcoholism: Clinical and Experimental Research 2019;43:1308-1317

Yambire KF, Fernandez-Mosquera L, Steinfeld R, Mühle C, Ikonen E, Milosevic I, Raimundo N: Mitochondrial biogenesis is transcriptionally repressed in lysosomal lipid storage diseases. eLife 2019;8.

Weinland C, Mühle C, Kornhuber J, Lenz B: Body mass index and craving predict 24-month hospital readmissions of alcohol-dependent in-patients following withdrawal. Progress in neuro-psychopharmacology & biological psychiatry 2019;90:300-307.

Wagner CJ, Musenbichler C, Böhm L, Färber K, Fischer A-I, von Nippold F, Winkelmann M, Mühle C, Kornhuber J, Lenz B: LDL cholesterol relates to depression, its severity, and the prospective course. Progress in neuro-psychopharmacology & biological psychiatry 2019

Mühle C, Barry B, Weinland C, Kornhuber J, Lenz B: Estrogen receptor 1 gene variants and estradiol activities in alcohol dependence. Progress in neuro-psychopharmacology & biological psychiatry 2019

Lenz B, Rother M, Bouna-Pyrrou P, Mühle C, Tektas OY, Kornhuber J: The androgen model of suicide completion. Progress in neurobiology 2019;172:84-103.

Mühle C, Weinland C, Gulbins E, Lenz B*, Kornhuber J*: Peripheral acid sphingomyelinase activity is associated with biomarkers and phenotypes of alcohol use and dependence in patients and healthy controls. International journal of molecular sciences 2018;19

Lenz B, Eichler A, Schwenke E, Buchholz VN, Hartwig C, Moll GH, Reich K, Mühle C, Volz B, Titzmann A, Beckmann MW, Heinrich H, Kornhuber J, Fasching PA: Mindfulness-based stress reduction in pregnancy: An app-based programme to improve the health of mothers and children (mindful/pmi study). Geburtshilfe und Frauenheilkunde 2018;78:1283-1291.

Lenz B*, Bouna-Pyrrou P*, Mühle C, Kornhuber J: Low digit ratio (2D:4D) and late pubertal onset indicate prenatal hyperandrogenziation in alcohol binge drinking. Progress in neuro-psychopharmacology & biological psychiatry 2018;86:370-378.

Huber SE, Zoicas I, Reichel M, Mühle C, Buttner C, Ekici AB, Eulenburg V, Lenz B*, Kornhuber J*, Müller CP*: Prenatal androgen receptor activation determines adult alcohol and water drinking in a sex-specific way. Addiction biology 2018;23:904-920.

Gegenhuber B, Weinland C, Kornhuber J, Mühle C*, Lenz B*: OPRM1 A118G and serum beta-endorphin interact with sex and digit ratio (2D:4D) to influence risk and course of alcohol dependence. European neuropsychopharmacology : the journal of the European College of Neuropsychopharmacology 2018;28:1418-1428. * contributed equally

Bouna-Pyrrou P, Aufleger B, Braun S, Gattnar M, Kallmayer S, Wagner H, Kornhuber J, Mühle C*, Lenz B*: Cross-sectional and longitudinal evaluation of the social network use disorder and internet gaming disorder criteria. Frontiers in psychiatry 2018;9:692. * contributed equally

Weinland C, Braun B, Mühle C, Kornhuber J, Lenz B: Cloninger type 2 score and Lesch typology predict hospital readmission of female and male alcohol-dependent inpatients during a 24-month follow-up. Alcohol Clin Exp Res 2017;41:1760-1767.

Rhein C, Reichel M, Kramer M, Rotter A, Lenz B, Mühle C, Gulbins E, Kornhuber J: Alternative splicing of SMPD1 coding for acid sphingomyelinase in major depression. Journal of affective disorders 2017;209:10-15.

Mühle C, Kornhuber J: Assay to measure sphingomyelinase and ceramidase activities efficiently and safely. Journal of chromatography A 2017;1481:137-144.

Lenz B, Mühle C, Braun B, Weinland C, Bouna-Pyrrou P, Behrens J, Kubis S, Mikolaiczik K, Muschler MR, Saigali S, Sibach M, Tanovska P, Huber SE, Hoppe U, Eichler A, Heinrich H, Moll GH, Engel A, Goecke TW, Beckmann MW, Fasching PA, Müller CP, Kornhuber J: Prenatal and adult androgen activities in alcohol dependence. Acta psychiatrica Scandinavica 2017;136:96-107.

Müller CP, Kalinichenko LS, Tiesel J, Witt M, Stockl T, Sprenger E, Fuchser J, Beckmann J, Praetner M, Huber SE, Amato D, Mühle C, Buttner C, Ekici AB, Smaga I, Pomierny-Chamiolo L, Pomierny B, Filip M, Eulenburg V, Gulbins E, Lourdusamy A, Reichel M, Kornhuber J: Paradoxical antidepressant effects of alcohol are related to acid sphingomyelinase and its control of sphingolipid homeostasis. Acta neuropathologica 2016

Mühle C, Kreczi J, Rhein C, Richter-Schmidinger T, Alexopoulos P, Doerfler A, Lenz B*, Kornhuber J*: Additive sex-specific influence of common non-synonymous DISC1 variants on amygdala, basal ganglia, and white cortical surface area in healthy young adults. Brain structure & function 2016

Lenz B, Thiem D, Bouna-Pyrrou P, Mühle C, Stoessel C, Betz P, Kornhuber J: Low digit ratio (2D:4D) in male suicide victims. J Neural Transm (Vienna) 2016;123:1499-1503.

Huston JP*, Kornhuber J*, Mühle C*, Japtok L, Komorowski M, Mattern C, Reichel M, Gulbins E, Kleuser B, Topic B, De Souza Silva MA, Müller CP: A sphingolipid mechanism for behavioral extinction. Journal of neurochemistry 2016 * contributed equally

Rhein C, Mühle C, Kornhuber J, Reichel M: Alleged detrimental mutations in the SMPD1 gene in patients with niemann-pick disease. International journal of molecular sciences 2015;16:13649-13652.

Müller CP, Reichel M, Mühle C, Rhein C, Gulbins E, Kornhuber J: Brain membrane lipids in major depression and anxiety disorders. Biochimica et biophysica acta 2015;1851:1052-1065.

Kornhuber J, Rhein C, Müller CP, Mühle C: Secretory sphingomyelinase in health and disease. Biological chemistry 2015;396:707-736.

Bouna-Pyrrou P*, Mühle C*, Kornhuber J, Lenz B: Internet gaming disorder, social network disorder and laterality: Handedness relates to pathological use of social networks. J Neural Transm (Vienna) 2015;122:1187-1196. * contributed equally

Schaller G, Sperling W, Richter-Schmidinger T, Mühle C, Heberlein A, Maihofner C, Kornhuber J, Lenz B: Serial repetitive transcranial magnetic stimulation (rTMS) decreases BDNF serum levels in healthy male volunteers. J Neural Transm 2014;121:307-313.

Rhein C, Reichel M, Mühle C, Rotter A, Schwab SG, Kornhuber J: Secretion of acid sphingomyelinase is affected by its polymorphic signal peptide. Cell Physiol Biochem 2014;34:1385-1401.

Rhein C, Mühle C, Richter-Schmidinger T, Alexopoulos P, Doerfler A, Kornhuber J: Neuroanatomical correlates of intelligence in healthy young adults: The role of basal ganglia volume. PLoS One 2014;9:e93623.

Reichel M, Richter-Schmidinger T, Mühle C, Rhein C, Alexopoulos P, Schwab SG, Gulbins E, Kornhuber J: The common acid sphingomyelinase polymorphism p.G508R is associated with self-reported allergy. Cell Physiol Biochem 2014;34:82-91.

Mühle C, Amova V, Biermann T, Bayerlein K, Richter-Schmidinger T, Kraus T, Reichel M, Gulbins E, Kornhuber J: Sex-dependent decrease of sphingomyelinase activity during alcohol withdrawal treatment. Cell Physiol Biochem 2014;34:71-81.

Grammatikos G, Mühle C, Ferreiros N, Schroeter S, Bogdanou D, Schwalm S, Hintereder G, Kornhuber J, Zeuzem S, Sarrazin C, Pfeilschifter J: Serum acid sphingomyelinase is upregulated in chronic hepatitis c infection and non alcoholic fatty liver disease. Biochimica et biophysica acta 2014;1841:1012-1020.

Rhein C, Naumann J, Mühle C, Zill P, Adli M, Hegerl U, Hiemke C, Mergl R, Moller HJ, Reichel M, Kornhuber J: The acid sphingomyelinase sequence variant p.A487V is not associated with decreased levels of enzymatic activity. JIMD reports 2013;8:1-6.

Mühle C, Reichel M, Gulbins E, Kornhuber J: Sphingolipids in psychiatric disorders and pain syndromes. Handbook of experimental pharmacology 2013:431-456.

Mühle C, Huttner HB, Walter S, Reichel M, Canneva F, Lewczuk P, Gulbins E, Kornhuber J: Characterization of acid sphingomyelinase activity in human cerebrospinal fluid. PLoS One 2013;8:e62912.

Rhein C, Tripal P, Seebahn A, Konrad A, Kramer M, Nagel C, Kemper J, Bode J, Mühle C, Gulbins E, Reichel M, Becker CM, Kornhuber J: Functional implications of novel human acid sphingomyelinase splice variants. PLoS One 2012;7:e35467.

Reichel M, Beck J, Mühle C, Rotter A, Bleich S, Gulbins E, Kornhuber J: Activity of secretory sphingomyelinase is increased in plasma of alcohol-dependent patients. Alcohol Clin Exp Res 2011;35:1852-1859.

Pio SF, Mühle C, De Oliveira GC, Rezende SM: Detection of int1h-related inversion of the factor viii gene. Haemophilia 2011;17:313-314.

Kornhuber J, Muehlbacher M, Trapp S, Pechmann S, Friedl A, Reichel M, Mühle C, Terfloth L, Groemer TW, Spitzer GM, Liedl KR, Gulbins E, Tripal P: Identification of novel functional inhibitors of acid sphingomyelinase. PLoS One 2011;6:e23852.

Mühle A*, Mühle C*, Amann K, Dotsch J, Nusken KD, Boltze J, Schneider H: No juvenile arterial hypertension in sheep multiples despite reduced nephron numbers. Pediatr Nephrol 2010;25:1653-1661. * contributed equally

Kornhuber J, Tripal P, Reichel M, Mühle C, Rhein C, Muehlbacher M, Groemer TW, Gulbins E: Functional inhibitors of acid sphingomyelinase (FIASMAs): A novel pharmacological group of drugs with broad clinical applications. Cell Physiol Biochem 2010;26:9-20.

Schneider H, Mühle C: X-chromosomal insertions at a recurrent site causing ectodermal dysplasia. Eur J Dermatol 2009;19:178-179.

Kornhuber J, Reichel M, Tripal P, Groemer TW, Henkel AW, Mühle C, Gulbins E: The role of ceramide in major depressive disorder. Eur Arch Psychiatry Clin Neurosci 2009;259 Suppl 2:S199-204.

Schneider H, Muehle C: Gene symbol: LAMC2. Disease: Epidermolysis bullosa, herlitz. Hum Genet 2008;123:544.

Gelse K, Mühle C, Knaup K, Swoboda B, Wiesener M, Hennig F, Olk A, Schneider H: Chondrogenic differentiation of growth factor-stimulated precursor cells in cartilage repair tissue is associated with increased HIF-1alpha activity. Osteoarthritis Cartilage 2008;16:1457-1465.

Gelse K, Mühle C, Franke O, Park J, Jehle M, Durst K, Goken M, Hennig F, von der Mark K, Schneider H: Cell-based resurfacing of large cartilage defects: Long-term evaluation of grafts from autologous transgene-activated periosteal cells in a porcine model of osteoarthritis. Arthritis Rheum 2008;58:475-488.

Schneider H, Mühle C, Pacho F: Biological function of laminin-5 and pathogenic impact of its deficiency. Eur J Cell Biol 2007;86:701-717.

Mühle C, Zenker M, Chuzhanova N, Schneider H: Recurrent inversion with concomitant deletion and insertion events in the coagulation factor VIII gene suggests a new mechanism for X-chromosomal rearrangements causing hemophilia a. Hum Mutat 2007;28:1045.

Mühle C, Schneider H: Novel human pathological mutations. Gene symbol: LAMA3. Disease: Junctional epidermolysis bullosa. Hum Genet 2007;122:549.

Mühle C*, Neuner A*, Park J, Pacho F, Jiang Q, Waddington SN, Schneider H: Evaluation of prenatal intra-amniotic LAMB3 gene delivery in a mouse model of herlitz disease. Gene Ther 2006;13:1665-1676.

Schneider H, Muehle C: Gene symbol: KRT14. Disease: Epidermolysis bullosa simplex. Hum Genet 2005;117:298.

Schneider H, Muehle C: Gene symbol: LAMB3. Disease: Junctional epidermolysis bullosa, herlitz variant. Hum Genet 2005;118:545.

Mühle C, Jiang QJ, Charlesworth A, Bruckner-Tuderman L, Meneguzzi G, Schneider H: Novel and recurrent mutations in the laminin-5 genes causing lethal junctional epidermolysis bullosa: Molecular basis and clinical course of herlitz disease. Hum Genet 2005;116:33-42.

Jakob M, Mühle C, Park J, Weiss S, Waddington S, Schneider H: No evidence for germ-line transmission following prenatal and early postnatal aav-mediated gene delivery. J Gene Med 2005;7:630-637.

Mühle C, Schulz-Drost S, Khrenov AV, Saenko EL, Klinge J, Schneider H: Epitope mapping of polyclonal clotting factor viii-inhibitory antibodies using phage display. Thromb Haemost 2004;91:619-625.

Schneider H, Mühle C, Douar AM, Waddington S, Jiang QJ, von der Mark K, Coutelle C, Rascher W: Sustained delivery of therapeutic concentrations of human clotting factor IX–a comparison of adenoviral and aav vectors administered in utero. J Gene Med 2002;4:46-53.

Machova Z, Mühle C, Krauss U, Trehin R, Koch A, Merkle HP, Beck-Sickinger AG: Cellular internalization of enhanced green fluorescent protein ligated to a human calcitonin-based carrier peptide. Chembiochem 2002;3:672-677.

Schneider H, Groves M, Mühle C, Reynolds PN, Knight A, Themis M, Carvajal J, Scaravilli F, Curiel DT, Fairweather NF, Coutelle C: Retargeting of adenoviral vectors to neurons using the HC fragment of tetanus toxin. Gene Ther 2000;7:1584-1592.

Chao TI, Grosche J, Friedrich KJ, Biedermann B, Francke M, Pannicke T, Reichelt W, Wulst M, Mühle C, Pritz-Hohmeier S, Kuhrt H, Faude F, Drommer W, Kasper M, Buse E, Reichenbach A: Comparative studies on mammalian muller (retinal glial) cells. J Neurocytol 1997;26:439-454.

 

Dr. Martin Regensburger

Clinician Scientist, MD rotation position 2017

Department of Stem Cell Biology
Prof. Winner

 

2011 MD, University of Regensburg

2012 M.Sc. (Neurosciences), University of Regensburg

since 2011 Resident, Department of Neurology and Postdoctoral Fellow, Department of Stem Cell Biology, FAU Erlangen-Nürnberg

Research focus

Motor neuron diseases comprise a heterogeneous group of neurodegenerative diseases leading to progressive disability. Focusing on amyotrophic lateral sclerosis and hereditary spastic paraplegia, I aim to improve the understanding of the molecular basis of these diseases. To this end, I use patient-derived induced pluripotent stem cells (iPS) and the respective derived neuronal subtypes are analyzed on the cellular and molecular level.

Publications

Pozner T*, Regensburger M*, Engelhorn T, Winkler J, Winner B (2020) Janus-faced spatacsin (SPG11): involvement in neurodevelopment and multisystem neurodegeneration. Brain. BRAIN-2019-01856.R1. *equal contribution

Regensburger M (2020) Which kinds of fasciculations are missed by ultrasonography in ALS? Clin Neurophysiol 131: 237-238. doi: 10.1016/j.clinph.2019.09.011

Regensburger M, Minakaki G, Kettwig M, Eisenhut F, Haack T, Kohl Z, Winkler J (2020) Novel biallelic CTSD gene variants cause late-onset ataxia and retinitis pigmentosa. Movement Disorders. Mov. Disord. 2020 May 18. doi: 10.1002/mds.28106. Online ahead of print.

Pozner T*, Schray A*, Regensburger M, Lie DC, Schlötzer-Schrehardt U, Winkler J, Turan S, Winner B (2018) Tideglusib Rescues Neurite Pathology of SPG11 iPSC Derived Cortical Neurons. Front Neurosci 2018 Dec 6;12:43. doi: 10.3389/fnins.2018.00914.s001 *equal contribution

Pérez-Brangulí F*, Buchsbaum IY*, Pozner T*, Regensburger M, Fan W, Schray A, Börstler T, Mishra H, Gräf D, Kohl Z, Winkler J, Berninger B, Cappello S, Winner B (2018) Human SPG11 cerebral organoids reveal cortical neurogenesis impairment. Hum Mol Genet 2018 Nov 22. doi: 10.1093/hmg/ddy397 *equal contribution

Popp B, Krumbiegel M, Grosch J, Sommer A, Uebe S, Kohl Z, Plötz S, Farrell M, Trautmann U, Kraus C, Ekici, AB, Asadollahi R, Regensburger M, Günther K, Rauch A, Edenhofer F, Winkler J, Winner B, Reis A (2018) Need for high-resolution Genetic Analysis in iPSC: Results and Lessons from the ForIPS Consortium. Sci Rep 8, 17201. doi:10.1038/s41598-018-35506-0

Regensburger M, Weidner N, Kohl Z (2018). Motor neuron disease – clinical and genetic differential diagnosis. Nervenarzt, 89, 658–665. doi:10.1038/nn.4000.

Regensburger M, Mielenz D, Winner B (2018). Swiprosin-1/EFhd2 – another piece in the puzzle of tauopathy? Aging. doi:10.18632/aging.101431. Editorial.

Regensburger M, Prots I, Reimer D, Brachs S, Loskarn S, Lie DC, Mielenz D, Winner B (2018) Impact of Swiprosin-1/EFhd2 on adult hippocampal neurogenesis. Stem Cell Rep 10(2):347-355.

Regensburger M, Tenner F, Möbius C, Schramm A (2018) Detection radius of EMG for fasciculations: empiric study combining ultrasonography and electromyography. Clin Neurophysiol 129:487-493. doi: 10.1016/j.clinph.2017.10.037

Regensburger M, Schreglmann SR, Stoll S, Rockenstein E, Loskarn S, Wei Xiang, Masliah E, Winner B (2018) Oligomer-prone E57K- mutant alpha-synuclein exacerbates integration deficit of adult hippocampal newborn neurons in transgenic mice. Brain Struct Funct 223(3):1357-1368.

Schneider-Gold C, Dekomien G, Regensburger M, Schneider R, Trampe N, Krogias C, Lukas C, Bellenberg B (2017). Monozygotic twins with a new compound heterozygous SPG11 mutation and different disease expression. J Neurol Sci 381:265-268. doi: 10.1016/j.jns.2017.09.005

Regensburger M, Türk M, Pagenstecher A, Schröder R, Winkler J (2017). VCP-related multisystem-proteinopathy presenting as early-onset Parkinson’s disease. Neurology 89(7):746–8. doi: 10.1212/WNL.0000000000004240

Tenner F*, Regensburger M*, Schramm A, Söhle M, Schwarzkopf K, Zalevsky Z, Schmidt M (2017). Evaluation of a Laser-Based Sensor for the Diagnosis of Neurological Disorders. Conf Proc IEEE Eng Med Biol Soc 2017:4231–4. *contributed equally

Schreglmann SR, Regensburger M, Rockenstein E, Masliah E, Xiang W, Winkler J, Winner B (2015) The temporal expression pattern of alpha-Synuclein modulates olfactory neurogenesis in transgenic mice. PLoS One 10(5):e0126261.

Madzar D, Hagge M, Möller S, Regensburger M, Lee DH, Schwab S, Jantsch J (2015).Endogenous endophthalmitis complicating Streptococcus equi subspecies zooepidemicus meningitis: a case report. BMC Res Notes 8:184. doi: 10.1186/s13104-015-1133-9

Regensburger M, Huttner HB, Doerfler A, Schwab S, Staykov D (2014). Propofol-related urine discoloration in a patient with fatal atypical intracerebral hemorrhage treated with hypothermia. Springerplus 3:551. doi: 10.1186/2193-1801-3-551

Purohit P, Perez-Branguli F, Prots I, Borger E, Gunn-Moore F, Welzel O, Loy K, Wenzel E, Grömer TW, Brachs S, Holzer M, Buslei R, Fritsch K, Regensburger M, Böhm KJ, Winner B, Mielenz D (2014) The Ca2+ sensor protein Swiprosin-1/EFhd2 is a synaptic protein and involved in kinesin-mediated axonal transport in neurons. PLoS One 18;9(8):e103976.

Pérez-Brangulí F, Mishra HK, Prots I, Havlicek S, Kohl Z, Saul D, Rummel C, Dorca-Arevalo J, Regensburger M, Graef D, Sock E, Blasi J, Groemer TW, Schlötzer-Schrehardt U, Winkler J, Winner B (2014) Dysfunction of spatacsin leads to axonal pathology in SPG11-linked hereditary spastic paraplegia. Hum Mol Genet 23(18):4859-74.

Schlachetzki JCM, Marxreiter F, Regensburger M, Kulinich A, Winner B, Winkler J (2014) Increased tyrosine hydroxylase expression accompanied by glial changes within the non-lesioned hemisphere in the 6-hydroxydopamine model of Parkinson’s disease. Restor Neurol Neurosci 32(4):447-62.

Regensburger M, Prots I, Winner B (2014). Adult hippocampal neurogenesis in Parkinson’s disease: impact on neuronal survival and plasticity. Neural Plast 2014:454696. doi: 10.1155/2014/454696. Review.

Marxreiter F, Regensburger M, Winkler J (2013). Adult neurogenesis in Parkinson’s disease. Cell Mol Life Sci. 2013 Feb;70(3):459-73. doi: 10.1007/s00018-012-1062-x. Review.

Winner B*, Regensburger M*, Schreglmann S. Boyer L, Prots I, Rockenstein E, ManteM, Zhao C, Winkler J, Masliah E, Gage FH (2012) Role of α-synuclein in adult neurogenesis and neuronal maturation in the dentate gyrus. J Neurosci 32(47):16906-16; *contributed equally

Kohl Z, Regensburger M, Aigner R, Kandasamy M, Winner B, Aigner L, Winkler J (2010) Impaired adult olfactory bulb neurogenesis in the R6/2 mouse model of Huntington’s disease. BMC Neurosci 13;11:114.

Regensburger M, Kohl Z, Grundmann K, Winner B, Riess O, Winkler J (2009) Adult neurogenesis in transgenic animal models of DYT1 dystonia. Neuroreport 20(17):1529-33.

Dr. Simone Reiprich

Group Leader

Institute of Biochemistry
Chair of Biochemistry and Pathobiochemistry

2007 Diploma in Molecular Medicine, FAU Erlangen-Nürnberg

2010 PhD, FAU Erlangen-Nürnberg

2010 Postdoctoral Fellow, Institute of Biochemistry, FAU Erlangen-Nürnberg

2016 Group Leader, Institute of Biochemistry, FAU Erlangen-Nürnberg

Research focus

Myelination and accordingly the development of myelin-forming glial cells is a central process during embryogenesis and regeneration of the nervous system. My research focuses on the transcriptional control of gliogenesis and its modulation by micro-RNAs. Technical approaches include animal models and primary cell culture as well as molecular biochemistry.

Publications

Wittstatt J, Weider M, Wegner M, Reiprich S (2020) MicroRNA miR-204 regulates proliferation and differentiation of oligodendroglia in culture. Glia. doi: 10.1002/glia.23821

Wittstatt J, Reiprich S, Küspert M.: Crazy Little Thing Called Sox-New Insights in Oligodendroglial Sox Protein Function. Int J Mol Sci. 2019 Jun 2;20(11). pii: E2713. doi: 10.3390/ijms20112713. Review.

Cantone M*, Küspert M*, Reiprich S*, Lai X, Eberhardt M, Göttle P, Beyer F, Azim K, Küry P, Wegner M, Vera J. 2018. A gene regulatory architecture that controls region-independent dynamics of oligodendrocyte differentiation. Glia. Print in process.

Hoffmann A, Ettle B, Battis K, Reiprich S, Schlachetzki JCM, Masliah E, Wegner M, Kuhlmann T, Riemenschneider MJ, Winkler J. 2018. Oligodendroglial α-synucleinopathy driven neuroinflammation in multiple system atrophy. Brain Pathology. Epub ahead of print.

Jacob A*, Wüst HM*, Thalhammer JM, Fröb F, Küspert M, Reiprich S, Balta EA, Lie DC, Wegner M, Sock E. 2018. The transcription factor prospero homeobox protein 1 is a direct target of SoxC proteins during developmental vertebrate neurogenesis. Journal of Neurochemistry. 146(3):251-268.

Turnescu T, Arter J, Reiprich S, Tamm ER, Waisman A, Wegner M. 2018. Sox8 and Sox10 jointly maintain myelin gene expression in oligodendrocytes. Glia. 66(2):279-294.

Reiprich S*, Cantone M*, Weider M*, Baroti T, Wittstatt J, Schmitt C, Küspert M, Vera J, Wegner M. 2017. Transcription factor Sox10 regulates oligodendroglial Sox9 levels via microRNAs. Glia. 65(7):1089-1102.

Ettle B, Kerman BE, Valera E, Gillmann C, Schlachetzki JC, Reiprich S, Büttner C, Ekici AB, Reis A, Wegner M, Bäuerle T, Riemenschneider MJ, Masliah E, Gage FH, Winkler J. 2016. α-Synuclein-induced myelination deficit defines a novel interventional target for multiple system atrophy. Acta Neuropathologica. 132(1):59-75.

Reiprich S, Wegner M. 2015. From CNS stem cells to neurons and glia: Sox for everyone. Cell and Tissue Research. 359(1):111-24. Review.

Ettle B, Reiprich S, Deusser J, Schlachetzki JCM, Xiang W, Prots I, Masliah E, Winner B, Wegner M, Winkler J. 2014. Intracellular alpha-synuclein affects early maturation of primary oligodendrocyte progenitor cells. Molecular and Cellular Neuroscience. 62:68-78.

Mao YY, Reiprich S, Wegner M, Fritzsch B. 2014. Targeted deletion of Sox10 by Wnt1-cre defects neuronal migration and projection in the mouse inner ear. PLoS One. 9(4):e94580.

Hoffmann S, Hos D, Küspert M, Lang RA, Lovell-Badge R, Wegner M, Reiprich S. 2014. Stem cell factor Sox2 and its close relative Sox3 have differentiation functions in oligodendrocytes. Development. 141(1):39-50.

Reiprich S, Wegner M. 2014. Sox2: A multitasking networker. Neurogenesis. 1(1):e962391. Commentary.

Vogl MR*, Reiprich S*, Küspert M, Kosian T, Schrewe H, Nave KA, Wegner M. 2013. Sox10 cooperates with the mediator subunit 12 during terminal differentiation of myelinating glia. Journal of Neuroscience. 33(15):6679-90.

Weider M*, Reiprich S*, Wegner M. 2013. Sox appeal – Sox10 attracts epigenetic and transcriptional regulators in myelinating glia. Biological Chemistry. 394(12):1583-93. Review.

Wahlbuhl M, Reiprich S, Vogl MR, Bösl MR, Wegner M. 2012. Transcription factor Sox10 orchestrates activity of a neural crest-specific enhancer in the vicinity of its gene. Nucleic Acids Research. 40(1): 88-101.

Reiprich S, Kriesch J, Schreiner S, Wegner M. 2010. Activation of Krox20 gene expression by Sox10 in myelinating Schwann cells. Journal of Neurochemistry. 112(3): 744–754.

Wolf M, Lommes P, Sock E, Reiprich S, Friedrich RP, Kriesch J, Stolt CC, Bermingham Jr. JR, Wegner M. 2009. Replacement of related POU transcription factors leads to severe defects in mouse forebrain development. Developmental Biology. 332(2): 418–428.

Pla P, Hirsch MR, Le Crom S, Reiprich S, Harley VR, Goridis C. 2008. Identification of Phox2b-regulated genes by expression profiling of cranial motoneuron precursors. Neural Development. 3: 14.

Reiprich S, Stolt CC, Schreiner S, Parlato R, Wegner M. 2008. SoxE proteins are differentially required in mouse adrenal gland development. Molecular Biology of the Cell. 19(4): 1575–1586.

*these authors contributed equally to the work

Dr. Patrick Süß

Clinician Scientist, MD rotation position 2019

Department for Molecular Neurology
Prof. Jürgen Winkler

2017 Resident, Institute of Neuropathology, University of Freiburg

2018 MD, FAU Erlangen-Nürnberg

2018 Resident, Department of Psychiatry and Psychotherapy, University of Freiburg

2019 Resident and Postdoctoral fellow, Department of Molecular Neurology, FAU Erlangen-Nürnberg

Research focus

Microglia are the brain-resident myeloid cells and the main effector cells of the innate immune system in the central nervous system (CNS). Together with other myeloid cell populations, they substantially influence CNS functions under homeostasis as well as in neuroinflammatory and neurodegenerative disorders. Moreover, CNS myeloid cells dynamically respond to peripheral inflammatory challenges.

My research focuses on the influence of the peripheral immune system on CNS myeloid cells in health and disease. By using animal models of both inflammatory diseases like rheumatoid arthritis (RA) and neurodegenerative diseases, I aim to elucidate spatiotemporal differences in the vulnerability of the CNS towards peripheral inflammation and develop treatment approaches targeting CNS myeloid cells from the periphery.

Publications

Original articles

Süß P*, Hoffmann A*, Rothe T*, Ouyang Z, Baum W, Staszewski O, Schett G, Prinz M, Krönke G, Glass CK, Winkler J, Schlachetzki JCM. Chronic peripheral inflammation causes a region-specific myeloid response in the central nervous system (2020). Cell Rep. *equal contribution

Shemer A, Grozovski J, Tay TL, Tao J, Volaski A, Süß P, Ardura-Fabregat A, Gross-Vered M, Kim JS, David E, Chappell-Maor L, Thielecke L, Glass CK, Cornils K, Prinz M, Jung S (2018). Engrafted parenchymal brain macrophages differ from microglia in transcriptome, chromatin landscape and response to challenge. Nat Commun 9, 5206.

Süß P, Kalinichenko L, Baum W, Reichel M, Kornhuber J, Loskarn S, Ettle B, Distler JH, Schett G, Winkler J, Müller CP, Schlachetzki JC (2015). Hippocampal structure and function are maintained despite severe innate peripheral inflammation. Brain Behav Immun 49, 156-170.

 

Reviews and comments

Süß P, Schlachetzki JCM. Microglia in Alheimer’s Disease (2020). Curr Alzheimer Res. doi: 10.2174/1567205017666200212155234.

Süß P (2017). Remote Control: Impacts of Peripheral Tumor Necrosis Factor-Alpha on Alzheimer Disease-Related Pathology. J Neurosci 37, 8045-8047.

 

Case Reports

Endres D, Pruss H, Rauer S, Süß P, Venhoff N, Feige B, Schweizer T, Nickel K, Maier S, Egger K, Domschke K, Meyer PT, Tebartz van Elst L (2020) Probable Autoimmune Catatonia With Antibodies Against Cilia on Hippocampal Granule Cells and Highly Suspicious Cerebral FDG-Positron Emission Tomography Findings. Biol Psychiatry 87: e29-e31. doi: 10.1016/j.biopsych.2019.12.020

Endres D, Rauer S, Kern W, Venhoff N, Maier SJ, Runge K, Süß P, Feige B, Nickel K, Heidt T, Domschke K, Egger K, Prüss H, Meyer PT, Tebartz van Elst L (2019). Psychiatric Presentation of Anti-NMDA Receptor Encephalitis. Front Neurol 10, 1086.

Lüngen EM, Maier V, Venhoff N, Salzer U, Dersch R, Berger B, Riering AN, Nickel K, Fiebich BL, Süß P, Maier SJ, Egger K, Tebartz van Elst L, Endres D (2019). Systemic Lupus Erythematosus With Isolated Psychiatric Symptoms and Antinuclear Antibody Detection in the Cerebrospinal Fluid. Front Psychiatry 10, 226.

Endres D, Süß P, Maier SJ, Friedel E, Nickel K, Ziegler C, Fiebich BL, Glocker FX, Stock F, Lange T, Dacko M, Venhoff N, Erny D, Doostkam S, Komlosi K, Domschke K, Tebartz van Est L (2019). New variant of MELAS Syndrome with Executive Dysfunction, Heteroplasmic Point Mutation in the MT-ND4 Gene (m.12015T>C; p.Leu419Pro) and comorbid polyglandular Autoimmune Syndrome Type 2. Front Immunol 10, 412.

Süß P, Volz F, Lang C, Staszewksi O, Palmedo G, Taschner CA, Scheiwe C, Kurz P, Mentzel T, Prinz M (2018). A Case of Large Meningeal Epithelioid Hemangioendothelioma With WWTR1-CAMTA1 Gene Rearrangement and Slow Growth Over 15 Years. J Neuropathol Exp Neurol 77, 871-876.

Rauer S, Stork L, Urbach H, Stathi A, Marx A, Süß P, Prinz M, Brück W, Metz I (2018). Drug reaction with eosinophilia and systemic symptoms after daclizumab therapy. Neurology 91, e359-e363.

Taschner CA, Süß P, Volz F, Urbach H, Dovi-Akué P, Prinz M (2018). Freiburg Neuropathology Case Conference: A Painless Mass Lesion of the Parietal and Occipital Bones. Clin Neuroradiol 28, 301-306.

Taschner CA, Süß P, Hohenhaus M, Urbach H, Lützen N, Prinz M (2018). Freiburg Neuropathology Case Conference: Tumor Located in the Anterior Portion of the Third Ventricle. Clin Neuroradiol 28,139-143.

Taschner CA, Süß P, Sajonz B, Urbach H, Simon-Gabriel CP, Prinz M (2017). Freiburg Neuropathology Case Conference: Widespread White Matter Lesions in a Patient with Progressive Paraparesis and Cortical Blindness. Clin Neuroradiol 27, 245-250.

Dr. Sören Turan

Research fellow and Project Leader Bavarian Research Network ForInter

Institute of Biochemistry
Working Group: Molecular Medicine with focus on Molecular Imaging

2007 Diploma in Biology TU Braunschweig

2011 Dr. rer. nat, Medical School Hannover

2011- 2013 Postdoctoral Fellow, Medical School Hannover

2013- 2015 Postdoctoral Fellow, Stanford University

2016 Postdoctoral Fellow, FAU Erlangen-Nürnberg

2019 Project Leader Bavarian Research Network ForInter

Research focus

Sox11 is a member of the group C SOX transcription factor family and is a critical transcriptional regulator of embryonic and adult neurogenesis. It was shown that heterozygous de novo mutations in Sox11 or deletions of Sox11 cause Coffin-Siris syndrome (CSS), a rare monogenic disease characterized by hypoplastic nails, microcephaly, and mental retardation. I am interested in developing and testing genetically engineered in vitro models to understand the role of Sox11 in human neurodevelopmental diseases.

Publications

Boerstler T, Wend H, Krumbiegel M, Kavyanifar A, Reis A, Lie CD, Winner B, Turan S (2020). CRISPR/Cas9 mediated generation of human ARID1B heterozygous knockout hESC lines to model Coffin-Siris syndrome. Stem Cell Res. 2020 Jun 29;47:101889. doi: 10.1016/j.scr.2020.101889.

Turan,S., Boerstler,T., Kavyanifar,A., Loskarn,S., Reis,A., Winner,B. and Lie,D.C. (2019) A novel human stem cell model for Coffin–Siris syndrome-like syndrome reveals the importance of SOX11 dosage for neuronal differentiation and survival. Hum. Mol. Genet., 28, 2589–2599.

Pozner, T*, Schray, A*, Regensburger, M, Lie, D.C., Schlötzer-Schrehardt, U, Winkler, J, Turan, S., Winner, B. Tideglusib Rescues Neurite Pathology of SPG11 iPSC Derived Cortical Neurons. Front Neurosci. (2018); 12:914. doi: 10.3389/fnins.2018.00914. *contributed equally

Kavyanifar, A.*, Turan, S.* & Lie, D.C. SoxC transcription factors: multifunctional regulators of neurodevelopment. Cell Tissue Res (2018) 371: 91. http://doi.org/10.1007/s00441-017-2708-7. *contributed equally

Turan, S., Farruggio, A. P., Srifa, W., Day, J. W., & Calos, M. P. Precise correction of disease mutations in induced pluripotent stem cells derived from patients with limb girdle muscular dystrophy. Molecular Therapy (2016) 24:4, 685-696

Geisinger, J. M., Turan, S., Hernandez, S., Spector, L. P., & Calos, M. P. In Vivo Blunt-End Cloning Through CRISPR / Cas9-Facilitated Non-Homologous end-joining. Nucl. Acids Res., (2016) 44:8,doi:10.1093/nar/gkv1542

Mayrhofer, P., Kratzer, B., Sommeregger, W., Steinfellner, W., Reinhart, D., Mader, A., Turan, S. … Kunert, R. Accurate comparison of antibody expression levels by reproducible transgene targeting in engineered recombination-competent CHO cells. Applied Microbiology and Biotechnology (2014), doi:10.1007/s00253-014-6011-1

Turan, S., Qiao, J., Madden, S., Benham, C., Kotz, M., Schambach, A., and Bode, J. Expanding Flp-RMCE options: the potential of Recombinase Mediated Twin-Site Targeting (RMTT). Gene. (2014) doi:10.1016/j.gene.2014.06.002

Kuehle, J.*, Turan, S.*, Cantz, T., Hoffmann, D., Suerth, J. D., Maetzig, T., … Schambach, A. Modified lentiviral LTRs allow Flp recombinase-mediated cassette exchange and in vivo tracing of “factor-free” induced pluripotent stem cells. Molecular Therapy, (2014) 22:5, 919–28. doi:10.1038/mt.2014.4 *contributed equally

Maetzig, T., Kuehle, J., Schwarzer, A., Turan, S., Rothe, M., Chaturvedi, A., … Schambach, A. All-in-One inducible lentiviral vector systems based on drug controlled FLP recombinase. Biomaterials, (2014) 35:14, 4345–56. doi:10.1016/j.biomaterials.2014.01.057

Turan, S., Zehe, C., Kuehle, J., Qiao, J., and Bode, J. Recombinase-mediated cassette exchange (RMCE) – a rapidly-expanding toolbox for targeted genomic modifications. Gene, (2013) 515(1), 1–27. doi:10.1016/j.gene.2012.11.016

Turan, S., and Bode, J. Site-specific recombinases: from tag-and-target- to tag-and-exchange-based genomic modifications. FASEB J. (2013) 25:4088-4107; doi:10.1096/fj.11-186940

Turan, S., Galla, M., Ernst, E., Qiao, J., Voelkel, C., Schiedlmeier, B., Zehe, C. and Bode, J. Recombinase-mediated cassette exchange (RMCE): traditional concepts and current challenges, Journal of Molecular Biology, (2011) 407(2):193-221

Turan, S., Kuehle, J., Schambach, A., Baum, C., Bode, J. Multiplexing RMCE: Versatile Extensions ofthe Flp-Recombinase-Mediated Cassette-Exchange Technology, Journal of Molecular Biology, (2010) 402:52-69, ISSN 0022-2836