Publications

Journal Article

• Georg Werner, Markus Damme, Martin Schludi, Johannes Gnörich, Karin Wind, Katrin Fellerer, Benedikt Wefers, Wolfgang Wurst, Dieter Edbauer, Matthias Brendel, Christian Haass, Anja Capell (2020)
  Loss of TMEM106B potentiates lysosomal and FTLD-like pathology in progranulin-deficient mice
  EMBO Rep. 2020 Oct 5;21(10):e50241.
• Yoshihiro Nihei, Kohji Mori, Georg Werner, Thomas Arzberger, Qihui Zhou, Barham Khosravi, Julia Japtok, Andreas Hermann, Andreas Sommacal, Markus Weber, German Consortium for Frontotemporal Lobar Degeneration, Bavarian Brain Banking Alliance, Frits Kamp, Brigitte Nuscher, Dieter Edbauer, Christian Haass (2020)
  Poly-glycine-alanine exacerbates C9orf72 repeat expansion-mediated DNA damage via sequestration of phosphorylated ATM and loss of nuclear hnRNPA3
  Acta Neuropathol 2020 Jan;139(1):99-118.
• Samira Parhizkar, Thomas Arzberger, Matthias Brendel, Gernot Kleinberger, Maximilian Deussing, Carola Focke, Brigitte Nuscher, Monica Xiong, Alireza Ghasemigharagoz, Natalie Katzmarski, Susanne Krasemann, Stefan F. Lichtenthaler, Stephan A. Müller, Alessio Colombo, Laura Sebastian Monasor, Sabina Tahirovic, Jochen Herms, Michael Willem, Nadine Pettkus, Oleg Butovsky, Peter Bartenstein, Dieter Edbauer, Axel Rominger, Ali Ertürk, Stefan A. Grathwohl, Jonas J. Neher, David M. Holtzman, Melanie Meyer-Luehmann, Christian Haass (2019)
  Loss of TREM2 function increases amyloid seeding but reduces plaque-associated ApoE
  Nat Neurosci. 2019 Feb;22(2):191-204
• Yu Ohki, Andrea Wenninger-Weinzierl, Alexander Hruscha, Kazuhide Asakawa, Koichi Kawakami, Christian Haass, Dieter Edbauer, Bettina Schmid (2017)
  Glycine-alanine dipeptide repeat protein contributes to toxicity in a zebrafish model of C9orf72 associated neurodegeneration
  Mol Neurodegener. 2017 Jan 14;12(1):6. doi: 10.1186/s13024-016-0146-8.
• Marc Suárez-Calvet, Manuela Neumann, Thomas Arzberger, Claudia Abou-Ajram, Eva Funk, Hannelore Hartmann, Dieter Edbauer, Elisabeth Kremmer, Christoph Göbl, Moritz Resch, Benjamin Bourgeois, Tobias Madl, Stefan Reber, Daniel Jutzi, Marc-David Ruepp, Ian R. A. Mackenzie, Olaf Ansorge, Dorothee Dormann, Christian Haass (2016)
  Monomethylated and unmethylated FUS exhibit increased binding to Transportin and distinguish FTLD-FUS from ALS-FUS
  Acta Neuropathol. 2016 Apr;131(4):587-604
• Kohji Mori, Yoshihiro Nihei, Thomas Arzberger, Qihui Zhou, Ian R Mackenzie, Andreas Hermann, Frank Hanisch, German Consortium for Frontotemporal Lobar Degeneration, Bavarian Brain Banking Alliance, Frits Kamp, Brigitte Nuscher, Denise Orozco, Dieter Edbauer, Christian Haass (2016)
  Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition
  EMBO Reports, published online 26.07.2016
• Fleck D, Voss M, Brankatschk B, Giudici C, Hampel H, Edbauer D, Steiner H, Kremmer E, Haug-Kröper M, Rossner MJ, Fluhrer R, Willem M, Haass C (2015)
  Proteolytic processing of neuregulin 1 type III by three intramembrane cleaving proteases
  J Biol Chem. 2016 Jan 1;291(1):318-33.
• Schwenk BM, Lang CM, Hogl S, Tahirovic S, Orozco D, Rentzsch K, Lichtenthaler SF, Hoogenraad CC, Capell A, Haass C, Edbauer D (2014)
  The FTLD risk factor TMEM106B and MAP6 control dendritic trafficking of lysosomes
  EMBO J. 2014 Jan 8. [Epub ahead of print]
• Mori K, Arzberger T, Grässer FA, Gijselinck I, Rentzsch K, Weng SM, Schludi HM, van der Zee J, Cruts M, Van Broeckhoven C, Kremmer E, Kretzschmar HA, Haass C, Edbauer D (2013)
  Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins.
  Acta Neuropathol. 2013 Dec;126(6):881-93. doi: 10.1007/s00401-013-1189-3. Epub 2013 Oct 17.
• Mackenzie IR, Arzberger T, Kremmer E, Troost D, Lorenzl S, Mori K, Weng SM, Haass C, Kretzschmar HA, Edbauer D, Neumann M (2013)
  Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations
  Acta Neuropathol. 2013 Dec;126(6):859-79. doi: 10.1007/s00401-013-1181-y. Epub 2013 Oct 6.
• Fleck D, van Bebber F, Colombo A, Galante C, Rabe L, Hampel H, Novak B, Kremmer E, Tahirovic S, Edbauer D, Lichtenthaler SF, Schmid B, Willem M, Haass C (2013)
  Dual cleavage of neuregulin 1 Type III by BACE1 and ADAM17 Liberates Its EGF-Like Domain and Allows Paracrine Signaling
  J Neurosci. 2013 May 1;33(18):7856-69. doi: 10.1523/JNEUROSCI.3372-12.2013
• Mori K, Lammich S, Mackenzie IR, Forne I, Zilow S, Kretzschmar H, Edbauer D, Janssens J, Kleinberger G, Cruts M, Herms J, Neumann M, Van Broeckhoven C, Arzberger T, Haass C (2013)
  hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations
  Acta Neuropathol. 2013 Mar;125(3):413-23. doi: 10.1007/s00401-013-1088-7. Epub 2013 Feb 5
• Schmid B, Hruscha A, Hogl S, Banzhaf-Strathmann J, van der Zee J, Teucke M, Eimer S, Hegermann J, Kittelmann M, Kremmer E, Cruts M, Solchenberger B, van Bebber F, Van Broeckhoven C, Edbauer D, Lichtenthaler SF, Haass C (2013)
  Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth
  Proc Natl Acad Sci U S A. 2013 Mar 1. [Epub ahead of print]
• Mori K, Weng SM, Arzberger T, Rentzsch K, Kremmer E, Schmid B, Kretzschmar HA, Cruts M, Van Broeckhoven C, Haass C, Edbauer D (2013)
  The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS
  Science. 2013 Feb 7. [Epub ahead of print]
• Orozco D, Tahirovic S, Rentzsch K, Haass C, Edbauer D (2012)
  Loss of fused in sarcoma (FUS) promotes pathological Tau splicing
  EMBO Rep. 2012 Jun 19. doi: 10.1038/embor.2012.90. [Epub ahead of print]
• Lang CM, Fellerer K, Schwenk BM, Kuhn PH, Kremmer E, Edbauer D, Capell A, Haass C (2012)
  Membrane orientation and subcellular localization of transmembrane protein 106B (TMEM106B), a major risk factor for frontotemporal lobar degeneration
  J Biol Chem. 2012 Apr 17. [Epub ahead of print]
• Dormann D, Rodde R, Edbauer D, Bentmann E, Fischer I, Hruscha A, Than ME, Mackenzie IR, Capell A, Schmid B, Neumann M, Haass C (2010)
  ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import
  EMBO J. 2010 Aug 18;29(16):2841-57. Epub 2010 Jul 6.
• Edbauer D, Kaether C, Steiner H, Haass C (2004)
  Co-expression of nicastrin and presenilin rescues a loss of function mutant of APH-1
  J Biol Chem 279(36): 37311-5
• Shirotani K, Edbauer D, Prokop S, Haass C, Steiner H (2004)
  Identification of distinct γ-secretase complexes with different APH-1 variants
  J Biol Chem 279(40): 41340-5
• Shirotani K, Edbauer D, Kostka M, Steiner H, Haass C (2004)
  Immature nicastrin stabilizes APH-1 independent of PEN-2 and presenilin: identification of nicastrin mutants that selectively interact with APH-1
  J Neurochem 89(6): 1520-7
• Prokop S, Shirotani K, Edbauer D, Haass C, Steiner H (2004)
  Requirement of PEN-2 for stabilization of the presenilin N-/C-terminal fragment heterodimer within the γ-secretase complex
  J Biol Chem 279(22): 23255-61
• Kaether C, Capell A, Edbauer D, Winkler E, Novak B, Steiner H, Haass C (2004)
  The presenilin C-terminus is required for ER-retention, nicastrin-binding and γ-secretase activity
  EMBO J 23(24): 4738-48
• Capell A, Kaether C, Edbauer D, Shirotani K, Merkl S, Steiner H, Haass C (2003)
  Nicastrin interacts with γ-secretase complex components via the N-terminal part of its transmembrane domain
  J Biol Chem 278(52): 52519-23
• Edbauer D, Winkler E, Regula JT, Pesold B, Steiner H, Haass C (2003)
  Reconstitution of γ-secretase activity
  Nat Cell Biol 5(5): 486-8
• Shirotani K, Edbauer D, Capell A, Schmitz J, Steiner H, Haass C (2003)
  γ-Secretase activity is associated with a conformational change of nicastrin
  J Biol Chem 278(19): 16474-7
• Edbauer D, Willem M, Lammich S, Steiner H, Haass C (2002)
  Insulin-degrading enzyme rapidly removes the β-amyloid precursor protein intracellular domain (AICD)
  J Biol Chem 277(16): 13389-93
• Steiner H, Winkler E, Edbauer D, Prokop S, Basset G, Yamasaki A, Kostka M, Haass C (2002)
  PEN-2 is an integral component of the γ-secretase complex required for coordinated expression of presenilin and nicastrin
  J Biol Chem 277(42): 39062-5
• Kaether C, Lammich S, Edbauer D, Ertl M, Rietdorf J, Capell A, Steiner H, Haass C (2002)
  Presenilin-1 affects trafficking and processing of βAPP and is targeted in a complex with nicastrin to the plasma membrane
  J Cell Biol 158(3): 551-61
• Moehlmann T, Winkler E, Xia X, Edbauer D, Murrell J, Capell A, Kaether C, Zheng H, Ghetti B, Haass C, Steiner H (2002)
  Presenilin-1 mutations of leucine 166 equally affect the generation of the Notch and APP intracellular domains independent of their effect on Aβ 42 production
  Proc Natl Acad Sci U S A 99(12): 8025-30
• Edbauer D, Winkler E, Haass C, Steiner H (2002)
  Presenilin and nicastrin regulate each other and determine amyloid β-peptide production via complex formation
  Proc Natl Acad Sci U S A 99(13): 8666-71
• Lammich S, Okochi M, Takeda M, Kaether C, Capell A, Zimmer AK, Edbauer D, Walter J, Steiner H, Haass C (2002)
  Presenilin-dependent intramembrane proteolysis of CD44 leads to the liberation of its intracellular domain and the secretion of an Aβ-like peptide
  J Biol Chem 277(47): 44754-9

Review

• Dieter Edbauer, Christian Haass (2016)
  An amyloid-like cascade hypothesis for C9orf72 ALS/FTD
  Curr Opin Neurobiol. 2016 Feb;36:99-106