Publications

Journal Article

• Marvin Reich, Matthew J Simon, Beate Polke, Iñaki Paris, Georg Werner, Christian Schrader, Lena Spieth, Sonnet S Davis, Sophie Robinson, Gabrielly Lunkes de Melo, Lennart Schlaphoff, Katrin Buschmann, Stefan Berghoff, Todd Logan, Brigitte Nuscher , Lis de Weerd, Dieter Edbauer, Mikael Simons, Jung H Suh, Thomas Sandmann, Mihalis S Kariolis, Sarah L DeVos, Joseph W Lewcock, Dominik Paquet , Anja Capell , Gilbert Di Paolo, Christian Haass (2024)
  Peripheral expression of brain-penetrant progranulin rescues pathologies in mouse models of frontotemporal lobar degeneration
  Sci Transl Med. 2024 Jun 5;16(750):eadj7308.
• Anika Reifschneider, Sophie Robinson, Bettina van Lengerich, Johannes Gnörich, Todd Logan, Steffanie Heindl, Miriam A Vogt, Endy Weidinger, Lina Riedl, Karin Wind, Artem Zatcepin, Ida Pesämaa, Sophie Haberl, Brigitte Nuscher, Gernot Kleinberger, Julien Klimmt, Julia K Götzl, Arthur Liesz, Katharina Bürger, Matthias Brendel, Johannes Levin, Janine Diehl-Schmid, Jung Suh, Gilbert Di Paolo, Joseph W Lewcock, Kathryn M Monroe, Dominik Paquet, Anja Capell, Christian Haass (2022)
  Loss of TREM2 rescues hyperactivation of microglia, but not lysosomal deficits and neurotoxicity in models of progranulin deficiency
  EMBO J. 2022 Jan 12; e109108
• 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.
• Lüningschrör P, Werner G, Stroobants S, Kakuta S, Dombert B, Sinske D, Wanner R, Lüllmann-Rauch R, Wefers B, Wurst W, D'Hooge R, Uchiyama Y, Sendtner M, Haass C, Saftig P, Knöll B, Capell A, Damme M (2020)
  The FTLD Risk Factor TMEM106B Regulates the Transport of Lysosomes at the Axon Initial Segment of Motoneurons
  Cell Rep. 2020 Mar 10;30(10):3506-3519.
• Marc Suárez-Calvet, Estrella Morenas-Rodríguez, Gernot Kleinberger, Kai Schlepckow, Miguel Ángel Araque Caballero, Nicolai Franzmeier, Anja Capell, Katrin Fellerer, Brigitte Nuscher, Erden Eren, Johannes Levin, Yuetiva Deming, Laura Piccio, Celeste M. Karch, Carlos Cruchaga, Leslie M. Shaw, John Q. Trojanowski, Michael Weiner, Michael Ewers, Christian Haass, for the Alzheimer’s Disease Neuroimaging Initiative (2019)
  Early increase of CSF sTREM2 in Alzheimer's disease is associated with tau related-neurodegeneration but not with amyloid-β pathology
  Mol Neurodegener. 2019 Jan 10;14(1):1
• Götzl J, Brendel M, Werner G, Parhizkar S, Sebastian Monasor L, Kleinberger G, Colombo A, Deussing M, Wagner M, Winkelmann J, Diehl-Schmid J, Levin J, Fellerer K, Reifschneider A, Bultmann S, Bartenstein P, Rominger A, Tahirovic S, Smith ST, Madore C, Butovsky O, Capell A, Haass C (2019)
  Opposite microglial activation stages upon loss of PGRN or TREM2 result in reduced cerebral glucose metabolism
  EMBO Mol Med. 2019 Jun;11(6):e9711.
• Marc Suárez-Calvet, Anja Capell, Miguel Ángel Araque Caballero, Estrella Morenas-Rodríguez, Katrin Fellerer, Nicolai Franzmeier, Gernot Kleinberger, Erden Eren, Yuetiva Deming, Laura Piccio, Celeste M Karch, Carlos Cruchaga, Katrina Paumier, Randall J Bateman, Anne M Fagan, John C Morris, Johannes Levin, Adrian Danek, Mathias Jucker, Colin L Masters, Martin N Rossor, John M Ringman, Leslie M Shaw, John Q Trojanowski, Michael Weiner, Michael Ewers , Christian Haass, for the Dominantly Inherited Alzheimer Network, & for the Alzheimer's Disease Neuroimaging Initiative (2018)
  CSF progranulin increases in the course of Alzheimer's disease and is associated with sTREM2, neurodegeneration and cognitive decline
  EMBO Mol Med. 2018 Dec;10(12). pii: e9712.
• Julia K. Götzl, Alessio-Vittorio Colombo, Katrin Fellerer, Anika Reifschneider, Georg Werner, Sabina Tahirovic, Christian Haass, Anja Capell (2018)
  Early lysosomal maturation deficits in microglia triggers enhanced lysosomal activity in other brain cells of progranulin knockout mice
  Mol Neurodegener. 2018 Sep 4;13(1):48
• Cornelis Blauwendraat, Carlo Wilke, Javier Simón-Sánchez, Iris E. Jansen, Anika Reifschneider, Anja Capell, Christian Haass, Melissa Castillo-Lizardo, Saskia Biskup, Walter Maetzler, Patrizia Rizzu, Peter Heutink, Matthis Synofzik (2018)
  The wide genetic landscape of clinical frontotemporal dementia: systematic combined sequencing of 121 consecutive subjects
  Genet Med. 2018 Feb;20(2):240-249
• Fargol Mazaheri, Nicolas Snaidero, Gernot Kleinberger, Charlotte Madore, Anna Daria, Georg Werner, Susanne Krasemann, Anja Capell, Dietrich Trümbach, Wolfgang Wurst, Bettina Brunner, Sebastian Bultmann, Sabina Tahirovic, Martin Kerschensteiner, Thomas Misgeld, Oleg Butovsky, Christian Haass (2017)
  TREM2 deficiency impairs chemotaxis and microglial responses to neuronal injury
  EMBO Rep. 2017 Jul;18(7):1186-1198
• Gernot Kleinberger, Anja Capell, Nathalie Brouwers, Katrin Fellerer, Kristel Sleegers, Marc Cruts, Christina Van Broeckhoven, Christian Haass (2016)
  Reduced secretion and altered proteolytic processing caused by missense mutations in progranulin
  Neurobiol Aging. 2016 Mar;39:220.e17-26
• Xianyuan Xiang, Georg Werner, Bernd Bohrmann, Arthur Liesz, Fargol Mazaheri, Anja Capell, Regina Feederle, Irene Knuesel, Gernot Kleinberger, Christian Haass (2016)
  TREM2 deficiency reduces the efficacy of immunotherapeutic amyloid clearance
  EMBO Molecular Medicine (2016) e201606370, Published online 08.07.2016
• Götzl JK, Mori K, Damme M, Fellerer K, Tahirovic S, Kleinberger G, Janssens J, van der Zee J, Lang CM, Kremmer E, Martin JJ, Engelborghs S, Kretzschmar HA, Arzberger T, Van Broeckhoven C, Haass C, Capell A (2014)
  Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis.
  Acta Neuropathol. 2014 Mar 12. [Epub ahead of print]
• Capell A, Fellerer K, Haass C (2014)
  Progranulin transcripts with short and long 5’-untranslated regions (UTR) are differentially expressed via post transcriptional and translational repression
  J Biol Chem. 2014 Jul 23. pii: jbc.M114.560128. [Epub ahead of print]
• 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]
• Wils H, Kleinberger G, Pereson S, Janssens J, Capell A, Van Dam D, Cuijt I, Joris G, De Deyn PP, Haass C, Van Broeckhoven C, Kumar-Singh S (2012)
  Cellular ageing, increased mortality and FTLD-TDP-associated neuropathology in progranulin knockout mice
  J Pathol. 2012 Jun 25. doi: 10.1002/path.4043. [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]
• Yasmina Cuartero, Maravillas Mellado, Anja Capell, Manuel Álvarez-Dolado, Marcel Verges (2012)
  Retromer regulates postendocytic sorting of β-secretase in polarized Madin-Darby canine kidney cells
  Traffic. 2012 Oct;13(10):1393-410
• Capell A, Liebscher S, Fellerer K, Brouwers N, Willem M, Lammich S, Gijselinck I, Bittner T, Carlson AM, Sasse F, Kunze B, Steinmetz H, Jansen R, Dormann D, Sleegers K, Cruts M, Herms J, Van Broeckhoven C, Haass C. (2011)
  Rescue of Progranulin Deficiency Associated with Frontotemporal Lobar Degeneration by Alkalizing Reagents and Inhibition of Vacuolar ATPase
  J Neurosci. 2011 Feb 2;31(5):1885-1894.
• 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.
• Fassler M, Zocher M, Klare S, de la Fuente AG, Scheuermann J, Capell A, Haass C, Valkova C, Veerappan A, Schneider D, Kaether C (2009)
  Masking of Transmembrane-Based Retention Signals Controls ER Export of gamma-Secretase
  Traffic. 2010 Feb;11(2):250-8. Epub 2009 Nov 5.
• Dormann D, Capell A, Carlson AM, Shankaran SS, Rodde R, Neumann M, Kremmer E, Matsuwaki T, Yamanouchi K, Nishihara M, Haass C (2009)
  Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin
  J Neurochem. 2009 Aug;110(3):1082-94. Epub 2009 Jun 9.
• Shankaran SS, Capell A, Hruscha AT, Fellerer K, Neumann M, Schmid B, Haass C (2008)
  Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion
  J Biol Chem 283(3): 1744-53
• Wild-Bode C, Fellerer K, Kugler J, Haass C, Capell A (2006)
  A basolateral sorting signal directs ADAM10 to adherens junctions and is required for its function in cell migration
  J Biol Chem 281(33): 23824-9
• Capell A, Beher D, Prokop S, Steiner H, Kaether C, Shearman MS, Haass C (2005)
  γ-Secretase complex assembly within the early secretory pathway
  J Biol Chem 280(8): 6471-8
• Peters N, Opherk C, Zacherle S, Capell A, Gempel P, Dichgans M (2004)
  CADASIL-associated Notch3 mutations have differential effects both on ligand binding and ligand-induced Notch3 receptor signaling through RBP-Jk
  Exp Cell Res. 2004 Oct 1;299(2):454-64.
• 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
• 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
• Fluhrer R, Capell A, Westmeyer G, Willem M, Hartung B, Condron MM, Teplow DB, Haass C, Walter J (2002)
  A non-amyloidogenic function of BACE-2 in the secretory pathway
  J Neurochem 81(5): 1011-20
• Capell A, Meyn L, Fluhrer R, Teplow DB, Walter J, Haass C (2002)
  Apical sorting of beta-secretase limits amyloid beta-peptide production
  J Biol Chem 277(7): 5637-43
• 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
• 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
• Walter J, Fluhrer R, Hartung B, Willem M, Kaether C, Capell A, Lammich S, Multhaup G, Haass C (2001)
  Phosphorylation regulates intracellular trafficking of beta-secretase
  J Biol Chem 276(18): 14634-41
• Sastre M, Steiner H, Fuchs K, Capell A, Multhaup G, Condron MM, Teplow DB, Haass C (2001)
  Presenilin-dependent γ-secretase processing of β-amyloid precursor protein at a site corresponding to the S3 cleavage of Notch
  EMBO Rep 2(9): 835-41
• Okochi M, Eimer S, Bottcher A, Baumeister R, Romig H, Walter J, Capell A, Steiner H, Haass C (2000)
  A loss of function mutant of the presenilin homologue SEL-12 undergoes aberrant endoproteolysis in Caenorhabditis elegans and increases Aβ42 generation in human cells
  J Biol Chem 275(52): 40925-32
• Steiner H, Kostka M, Romig H, Basset G, Pesold B, Hardy J, Capell A, Meyn L, Grim ML, Baumeister R, Fechteler K, Haass C (2000)
  Glycine 384 is required for presenilin-1 function and is conserved in bacterial polytopic aspartyl proteases
  Nat Cell Biol 2(11): 848-51
• Capell A, Steiner H, Willem M, Kaiser H, Meyer C, Walter J, Lammich S, Multhaup G, Haass C (2000)
  Maturation and pro-peptide cleavage of β-secretase
  J Biol Chem 275(40): 30849-54
• Capell A, Steiner H, Romig H, Keck S, Baader M, Grim MG, Baumeister R, Haass C (2000)
  Presenilin-1 differentially facilitates endoproteolysis of the β-amyloid precursor protein and Notch
  Nat Cell Biol 2(4): 205-11
• Kulic L, Walter J, Multhaup G, Teplow DB, Baumeister R, Romig H, Capell A, Steiner H, Haass C (2000)
  Separation of presenilin function in amyloid β-peptide generation and endoproteolysis of Notch
  Proc Natl Acad Sci U S A 97(11): 5913-8
• Steiner H, Duff K, Capell A, Romig H, Grim MG, Lincoln S, Hardy J, Yu X, Picciano M, Fechteler K, Citron M, Kopan R, Pesold B, Keck S, Baader M, Tomita T, Iwatsubo T, Baumeister R, Haass C (1999)
  A loss of function mutation of presenilin-2 interferes with amyloid β-peptide production and notch signaling
  J Biol Chem 274(40): 28669-73
• Guenette SY, Chen J, Ferland A, Haass C, Capell A, Tanzi RE (1999)
  hFE65L influences amyloid precursor protein maturation and secretion
  J Neurochem 73(3): 985-93
• Capell A, Grunberg J, Pesold B, Diehlmann A, Citron M, Nixon R, Beyreuther K, Selkoe DJ, Haass C (1998)
  The proteolytic fragments of the Alzheimer's disease-associated presenilin-1 form heterodimers and occur as a 100-150-kDa molecular mass complex
  J Biol Chem 273(6): 3205-11
• Capell A, Saffrich R, Olivo JC, Meyn L, Walter J, Grunberg J, Mathews P, Nixon R, Dotti C, Haass C (1997)
  Cellular expression and proteolytic processing of presenilin proteins is developmentally regulated during neuronal differentiation
  J Neurochem 69(6): 2432-40
• Walter J, Capell A, Hung AY, Langen H, Schnolzer M, Thinakaran G, Sisodia SS, Selkoe DJ, Haass C (1997)
  Ectodomain phosphorylation of beta-amyloid precursor protein at two distinct cellular locations
  J Biol Chem 272(3): 1896-903
• Wild-Bode C, Yamazaki T, Capell A, Leimer U, Steiner H, Ihara Y, Haass C (1997)
  Intracellular generation and accumulation of amyloid β-peptide terminating at amino acid 42
  J Biol Chem 272(26): 16085-8
• Walter J, Grunberg J, Capell A, Pesold B, Schindzielorz A, Citron M, Mendla K, George-Hyslop PS, Multhaup G, Selkoe DJ, Haass C (1997)
  Proteolytic processing of the Alzheimer disease-associated presenilin-1 generates an in vivo substrate for protein kinase C
  Proc Natl Acad Sci U S A 94(10): 5349-54
• Tomita T, Maruyama K, Saido TC, Kume H, Shinozaki K, Tokuhiro S, Capell A, Walter J, Grunberg J, Haass C, Iwatsubo T, Obata K (1997)
  The presenilin 2 mutation (N141I) linked to familial Alzheimer disease (Volga German families) increases the secretion of amyloid beta protein ending at the 42nd (or 43rd) residue
  Proc Natl Acad Sci U S A 94(5): 2025-30
• Citron M, Diehl TS, Capell A, Haass C, Teplow DB, Selkoe DJ (1996)
  Inhibition of amyloid beta-protein production in neural cells by the serine protease inhibitor AEBSF
  Neuron 17(1): 171-9
• Capell A, Teplow DB, Citron M, Selkoe DJ, Haass C (1996)
  Substitution of phenylalanine by proline at position 19 of amyloid-ß peptide results in the inhibition of protein kinase C mediated decrease of amyloidogenic processing
  Amyloid 3; 150-155
• Walter J, Capell A, Grunberg J, Pesold B, Schindzielorz A, Prior R, Podlisny MB, Fraser P, Hyslop PS, Selkoe DJ, Haass C (1996)
  The Alzheimer's disease-associated presenilins are differentially phosphorylated proteins located predominantly within the endoplasmic reticulum
  Mol Med 2(6): 673-91
• Haass C, Koo EH, Capell A, Teplow DB, Selkoe DJ (1995)
  Polarized sorting of beta-amyloid precursor protein and its proteolytic products in MDCK cells is regulated by two independent signals
  J Cell Biol 128(4): 537-47
• Haass C, Lemere CA, Capell A, Citron M, Seubert P, Schenk D, Lannfelt L, Selkoe DJ (1995)
  ß-Secretase cleavage of ß-Amyloid precursor protein with the Swedish mutation occurs within the secretory pathway after the trans-Golgi network
  Nat. Med. 1; 1291-6
• Haass C, Capell A, Citron M, Teplow DB, Selkoe DJ (1995)
  The vacuolar H(+)-ATPase inhibitor bafilomycin A1 differentially affects proteolytic processing of mutant and wild-type beta-amyloid precursor protein
  J Biol Chem 270(11): 6186-92

Review

• Julia Götzl, Christina Lang, Christian Haass, Anja Capell (2016)
  Impaired protein degradation in FTLD and related disorders
  Ageing Res Rev. 2016 May 7. pii: S1568-1637(16)30065-4
• Kleinberger G, Capell A, Haass C, Van Broeckhoven C (2013)
  Mechanisms of granulin deficiency: lessons from cellular and animal models
  Mol Neurobiol. 2013 Feb;47(1):337-60. doi: 10.1007/s12035-012-8380-8. Epub 2012 Dec 13
• Steiner H, Capell A, Leimer U, Haass C (1999)
  Genes and mechanisms involved in β-amyloid generation and Alzheimer's disease
  Eur Arch Psychiatry Clin Neurosci 249(6): 266-70
• Steiner H, Capell A, Haass C (1999)
  Proteolytic processing and degradation of Alzheimer's disease relevant proteins
  Biochem Soc Trans 27(2): 234-42
• Haass C, Grunberg J, Capell A, Wild-Bode C, Leimer U, Walter J, Yamazaki T, Ihara I, Zweckbronner I, Jakubek C, Baumeister R (1998)
  Proteolytic processing of Alzheimer's disease associated proteins
  J Neural Transm Suppl 53: 159-67