Publications
Journal Article
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Werner NT, Högel P, Güner G, Stelzer W, Wozny M, Aßfalg M, Lichtenthaler SF, Steiner H, Langosch D
(2023)
Cooperation of N- and C-terminal substrate transmembrane domain segments in intramembrane proteolysis by γ-secretase
Commun Biol. 2023 Feb 15;6(1):177 -
Schmidt FC, Fitz K, Feilen LP, Okochi M, Steiner H, Langosch D
(2023)
Different transmembrane domains determine the specificity and efficiency of the cleavage activity of the γ-secretase subunit presenilin
J Biol Chem. 2023 Mar 20;299(5):104626. -
Dawkins E, Derks RJE, Schifferer M, Trambauer J, Winkler E, Simons M, Paquet D, Giera M, Kamp F, Steiner H
(2023)
Membrane lipid remodeling modulates γ-secretase processivity
J Biol Chem. 2023 Apr;299(4):103027. -
Feilen LP, Chen SY, Fukumori A, Feederle R, Zacharias M, Steiner H
(2022)
Active site geometry stabilization of a presenilin homolog by the lipid bilayer promotes intramembrane proteolysis
Elife. 2022 May 17;11:e76090. -
Güner G, Aßfalg M, Zhao K, Dreyer T, Lahiri S, Lo Y, Slivinschi BI, Imhof A, Jocher G, Strohm L, Behrends C, Langosch D, Bronger H, Nimsky C, Bartsch JW, Riddell SR, Steiner H, Lichtenthaler SF
(2022)
Proteolytically generated soluble Tweak Receptor Fn14 is a blood biomarker for γ-secretase activity
EMBO Mol Med. 2022 Oct 10;14(10):e16084 -
Junker J, Kamp F, Winkler E, Steiner H, Bracher F, Müller C
(2021)
Effective sample preparation procedure for the analysis of free neutral steroids, free steroid acids and sterol sulfates in different tissues by GC-MS
J Steroid Biochem Mol Biol. 2021 Jul;211:105880 -
Colombo A, Sadler RK, Llovera G, Singh V, Roth S, Heindl S, Sebastian Monasor L, Verhoeven A, Peters F, Parhizkar S, Kamp F, Gomez de Aguero M, MacPherson AJ, Winkler E, Herms J, Benakis C, Dichgans M, Steiner H, Giera M, Haass C, Tahirovic S, Liesz A
(2021)
Microbiota-derived short chain fatty acids modulate microglia and promote Aβ plaque deposition
Elife. 2021 Apr 13;10:e59826 -
Saretz S, Basset G, Useini L, Laube M, Pietzsch J, Draca D, Maksimovic-Ivanic D, Trambauer J, Steiner H, Hey-Hawkins E
(2021)
Modulation of γ-secretase activity by a carborane-based flurbiprofen analogue
Molecules. 2021 May 11;26(10):2843 -
Trambauer J, Rodríguez Sarmiento RM, Fukumori A, Feederle R, Baumann K, Steiner H
(2020)
Aβ43-producing PS1 FAD mutants cause altered substrate interactions and respond to γ-secretase modulation
EMBO Rep. 2020 Jan 7;21(1):e47996 -
Kong L, Dawkins E, Campbell F, Winkler E, Derks RJE, Giera M, Kamp F, Steiner H, Kros A
(2020)
Photo-controlled delivery of very long chain fatty acids to cell membranes and modulation of membrane protein function
Biochim Biophys Acta Biomembr. 2020 May 1;1862(5):183200 -
Steiner A, Schlepckow K, Brunner B, Steiner H, Haass C, Hagn F
(2020)
γ-Secretase cleavage of the Alzheimer risk factor TREM2 is determined by its intrinsic structural dynamics
EMBO J. 2020 Oct 15;39(20):e104247 -
Torben Mentrup, Kosta Theodorou, Florencia Cabrera-Cabrera, Andreas O. Helbig, Kathrin Happ, Marion Gijbels, Ann-Christine Gradtke, Björn Rabe, Akio Fukumori, Harald Steiner, Andreas Tholey, Regina Fluhrer, Marjo Donners, Bernd Schröder
(2019)
Atherogenic LOX-1 signaling is controlled by SPPL2-mediated intramembrane proteolysis
J Exp Med. 2019 Apr 1;216(4):807-830 -
Vöglein J, Paumier K, Jucker M, Preische O, McDade E, Hassenstab J, Benzinger TL, Noble JM, Berman SB, Graff-Radford NR, Ghetti B, Farlow MR, Chhatwal J, Salloway S, Xiong C, Karch CM, Cairns N, Mori H, Schofield PR, Masters CL, Goate A, Buckles V, Fox N, Rossor M, Chrem P, Allegri R, Ringman JM, Höglinger G, Steiner H, Dieterich M, Haass C, Laske C, Morris JC, Bateman RJ, Danek A, Levin J, Dominantly Inherited Alzheimer Network
(2019)
Clinical, pathophysiological and genetic features of motor symptoms in autosomal dominant Alzheimer's disease
Brain 2019 May 1;142(5):1429-1440 -
Junker J, Chong I, Kamp F, Steiner H, Giera M, Müller C, Bracher F
(2019)
Comparison of strategies for the determination of sterol sulfates via GC-MS leading to a novel deconjugation-derivatization protocol
Molecules. 2019 Jun 26;24(13):2353 -
Vöglein J, Willem M, Trambauer J, Schönecker S, Dieterich M, Biskup S, Giudici C, Utz K, Oberstein T, Brendel M, Rominger A, Danek A, Steiner H, Haass C, Levin J
(2019)
Identification of a rare presenilin 1 single amino acid deletion mutation (F175del) with unusual amyloid-β processing effects
Neurobiol Aging. 2019 Dec;84:241.e5-241.e11 -
Tretter JY, Schorpp K, Luxenburger E, Trambauer J, Steiner H, Hadian K, Gires O, Niessing D
(2018)
A high-content screen for small-molecule regulators of epithelial cell-adhesion molecule (EpCAM) cleavage yields a robust inhibitor
J Biol Chem. 2018 Jun 8;293(23):8994-9005 -
Frits Kamp, Holger A. Scheidt, Edith Winkler, Gabriele Basset, Hannes Heinel, James M. Hutchison, Loren M. LaPointe, Charles R. Sanders, Harald Steiner, Daniel Huster
(2018)
Bexarotene binds to the amyloid precursor protein transmembrane domain, alters its α-helical conformation, and inhibits γ-secretase nonselectively in liposomes
ACS Chem Neurosci. 2018 Jul 18;9(7):1702-1713. -
Alexander Götz, Nadine Mylonas, Philipp Högel, Mara Silber, Hannes Heinel, Simon Menig, Alexander Vogel, Hannes Feyrer, Daniel Huster, Burkhard Luy, Dieter Langosch, Christina Scharnagl, Claudia Muhle-Goll, Frits Kamp, Harald Steiner
(2018)
Modulating hinge flexibility in the APP transmembrane domain alters γ-secretase
Biophys J. 2019 Jun 4;116(11):2103-2120 -
Trambauer J, Fukumori A, Kretner B, Steiner H
(2017)
Analyzing amyloid-β peptide modulation profiles and binding sites of γ-secretase modulators
Methods Enzymol. 2017;584:157-183 -
Kai Schlepckow, Gernot Kleinberger, Akio Fukumori, Regina Feederle, Stefan F. Lichtenthaler, Harald Steiner, Christian Haass
(2017)
An Alzheimer‐associated TREM2 variant occurs at the ADAM cleavage site and affects shedding and phagocytic function
EMBO Mol Med. 2017 Oct;9(10):1356-1365 -
Benedikt Kretner, Johannes Trambauer, Janina Mielke, Peer-Hendrik Kuhn, Elisabeth Kremmer, Armin Giese, Stefan Lichtenthaler, Christian Haass, Thomas Arzberger, Harald Steiner
(2016)
Generation and deposition of Aβ43 by the virtually inactive presenilin-1 L435F mutant contradicts the presenilin loss-of-function hypothesis of Alzheimer’s disease
EMBO Mol Med. 2016 May 2;8(5):458-65 -
Susann Hüttl, Felix Helfrich, Torben Mentrup, Sebastian Held, Harald Steiner, Paul Saftig, Regina Fluhrer, Bernd Schröder
(2016)
Substrate determinants of signal peptide peptidase-like 2a (SPPL2a)-mediated intramembrane proteolysis of the invariant chain CD74
Biochem J. 2016 May 15;473(10):1405-22. -
M Brendel, A Jaworska, J Herms, J Trambauer, C Rötzer, F-J Gildehaus, J Carlsen, P Cumming, J Bylund, T Luebbers, P Bartenstein, H Steiner, C Haass, K Baumann, A Rominger
(2015)
Amyloid-PET predicts inhibition of de novo plaque formation upon chronic γ-secretase modulator treatment
Mol Psychiatry. 2015 Oct;20(10):1179-87 -
Matthias Brendel, Anna Jaworska, Eric Grießinger, Christina Rötzer, Steffen Burgold, Franz-Josef Gildehaus, Janette Carlsen, Paul Cumming, Karlheinz Baumann, Christian Haass, Harald Steiner, Peter Bartenstein, Jochen Herms, Axel Rominger
(2015)
Cross-sectional comparison of small animal [18F]-florbetaben amyloid-PET between transgenic AD mouse models
PLoS One. 2015 Feb 23;10(2):e0116678 -
Teresa Bachhuber, Natalie Katzmarski, Joanna F McCarter, Desiree Loreth, Sabina Tahirovic, Frits Kamp, Claudia Abou-Ajram, Brigitte Nuscher, Alberto Serrano-Pozo, Alexandra Müller, Marco Prinz, Harald Steiner, Bradley T Hyman, Christian Haass, Melanie Meyer-Luehmann
(2015)
Inhibition of amyloid-β plaque formation by α-synuclein
Nat Med. 2015 Jul;21(7):802-7 -
Frits Kamp, Edith Winkler, Johannes Trambauer, Amelie Ebke, Regina Fluhrer, Harald Steiner
(2015)
Intramembrane proteolysis of β-amyloid precursor protein by γ-secretase is an unusually slow process
Biophys J. 2015 Mar 10;108(5):1229-37. -
M Brendel, A Jaworska, J Herms, J Trambauer, C Rötzer, F-J Gildehaus, J Carlsen, P Cumming, J Bylund, T Luebbers, P Bartenstein, H Steiner, C Haass, K Baumann, A Rominger
(2015)
Monitoring of chronic γ-secretase modulator treatment by serial amyloid-PET
Mol Psychiatry. 2015 Oct;20(10):1141 -
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. -
Voss M, Künzel U, Higel F, Kuhn PH, Colombo A, Haug-Kröper M, Klier B, Grammer G, Seidl A, Schröder B, Obst R, Steiner H, Lichtenthaler SF, Haass C, Fluhrer R
(2014)
Shedding of glycan-modifying enzymes by signal peptide peptidase-like 3 (SPPL3) regulates cellular N-glycosylation
EMBO J. 2014 Dec 17;33(24):2890-905. doi: 10.15252/embj.201488375. Epub 2014 Oct 29. -
Liebscher S, Page RM, Käfer K, Winkler E, Quinn K, Goldbach E, Brigham EF, Quincy D, Basi GS, Schenk DB, Steiner H, Bonhoeffer T, Haass C, Meyer-Luehmann M, Hübener M
(2013)
Chronic γ-secretase inhibition reduces amyloid plaque-associated instability of pre- and postsynaptic structures.
Mol Psychiatry. 2013 Sep 24. doi: 10.1038/mp.2013.122. [Epub ahead of print] -
Brendel M, Delker A, Rötzer C, Böning G, Carlsen J, Cyran C, Mille E, Gildehaus FJ, Cumming P, Baumann K, Steiner H, Haass C, Herms J, Bartenstein P, Rominger A
(2013)
Impact of partial volume effect correction on cerebral β-amyloid imaging in APP-Swe mice using [(18)F]-florbetaben PET.
Neuroimage. 2014 Jan 1;84:843-53. doi: 10.1016/j.neuroimage.2013.09.017. Epub 2013 Sep 20. -
Kretner B, Kuhn PH, Pérez-Revuelta BI, Lichtenthaler SF, Haass C, Steiner H
(2013)
Important functional role of residue x of the presenilin GxGD protease active site motif for APP substrate cleavage specificity and substrate selectivity of γ-secretase
J Neurochem. 2013 Apr;125(1):144-56. doi: 10.1111/jnc.12124. Epub 2013 Jan 15 -
Rominger A, Brendel M, Burgold S, Keppler K, Baumann K, Xiong G, Mille E, Gildehaus FJ, Carlsen J, Schlichtinger J, Niedermoser S, Wängler B, Cumming P, Steiner H, Herms J, Haass C, Bartenstein P
(2013)
Longitudinal assessment of cerebral β-amyloid deposition in mice overexpressing Swedish mutant β-amyloid precursor protein using 18F-florbetaben PET.
J Nucl Med. 2013 Jul;54(7):1127-34. doi: 10.2967/jnumed.112.114660. Epub 2013 May 31. -
Voss M, Kuhn PH, Künzel U, Klier B, Haug-Kröper M, Kremmer E, Lichtenthaler SF, Steiner H, Schröder B, Fluhrer R
(2012)
Foamy virus envelope protein is a substrate for signal peptide peptidase-like 3 (SPPL3)
J Biol Chem. 2012 Dec 21;287(52):43401-9. doi: 10.1074/jbc.M112.371369. Epub 2012 Nov 6. -
Winkler E, Kamp F, Scheuring J, Ebke A, Steiner H
(2012)
Generation of Alzheimer disease-associated Aβ42/43 by γ-secretase can directly be inhibited by modulation of membrane thickness
J. Biol. Chem. 287, 21326-34. -
Page RM, Münch A, Horn T, Kuhn PH, Colombo A, Reiner O, Boutros M, Steiner H, Lichtenthaler SF, Haass C
(2012)
Loss of PAFAH1B2 reduces amyloid-β generation by promoting the degradation of amyloid precursor protein C-terminal fragments
J Neurosci. 2012 Dec 12;32(50):18204-18214. -
Kretner B, Gutsmiedl A, Page RM, Luebbers T, Galley G, Baumann KH, Haass C, Steiner H
(2011)
Attenuated Aβ42 responses to low potency γ-secretase modulators can be overcome for many pathogenic presenilin mutants by second-generation compounds
J Biol Chem. 2011 Apr 29;286(17):15240-51. Epub 2011 Feb 25. -
Ebke A, Luebbers T, Shirotani K, Haass C, Baumann KH, Steiner H
(2011)
Novel γ-secretase modulators directly target presenilin
J Biol Chem. 2011 Oct 28;286(43):37181-6. Epub 2011 Sep 6. -
Fluhrer R, Kamp F, Nuscher B, Steiner H, Beyer K, Haass C
(2011)
The nicastrin ectodomain adopts a highly thermostable structure
Biol Chem. 2011 Aug 18. [Epub ahead of print] -
Mitterreiter S, Page RM, Kamp F, Hopson J, Ha HR, Hamid R, Herms J, Mayer TU, Nelson DJ, Steiner H, Stahl T, Zeitschel U, Roßner S, Haass C
(2010)
Bepridil and Amiodarone Simultaneously Target the Alzheimer’s Disease β- and γ-Secretase via Distinct Mechanisms
J. Neurosci. 2010 Apr 20. [Epub ahead of print] -
Fluhrer R, Steiner H, Haass C
(2010)
Three-amino acid spacing of presenilin endoproteolysis suggests a general stepwise cleavage of γ-secretase-mediated intramembrane proteolysis
J. Neurosci. 2010 Apr 20. [Epub ahead of print] -
Höttecke N, Liebeck M, Baumann K, Schubenel R, Winkler E, Steiner H, Schmidt B
(2010)
Inhibition of γ-secretase by the CK1 inhibitor IC261 does not depend on CK1δ
Bioorg Med Chem Lett. 2010 Mar 4. [Epub ahead of print] -
Fluhrer R, Steiner H, Haass C
(2010)
Three-amino acid spacing of presenilin endoproteolysis suggests a general stepwise cleavage of γ-secretase-mediated intramembrane proteolysis
J Neurosci. 2010 Jun 9;30(23):7853-62. -
Page RM, Gutsmiedl A, Haass C, Steiner H
(2010)
β-Amyloid precursor protein mutants respond to γ-secretase modulators
J Biol Chem. 2010 Jun 4;285(23):17798-810. Epub 2010 Mar 26. -
Hobson S, Dümpelfeld B, Luebbers T, Baumann K, Haass C, Hopf C, Steiner H
(2009)
Purification, pharmacological modulation, and biochemical characterization of interactors of endogenous human γ-secretase
Biochemistry 2009 Jan 21. -
Pérez-Revuelta BI, Lammich S, Yamasaki A, Haass C, Steiner H
(2009)
Requirement for small side chain residues within the GxGD-motif of presenilin for gamma-secretase substrate cleavage
J Neurochem. 2010 Feb;112(4):940-50. Epub 2009 Dec 15. -
Bittner T, Fuhrmann M, Burgold S, Jung CK, Volbracht C, Steiner H, Mitteregger G, Kretzschmar HA, Haass C, Herms J
(2009)
γ-Secretase inhibition reduces spine density in vivo via an amyloid precursor protein-dependent pathway
J Neurosci. 2009 Aug 19;29(33):10405-9. -
Steiner H, Winkler E, Haass C
(2008)
Chemical cross-linking provides a model of the γ-secretase complex subunit architecture and evidence for close proximity of the C-terminal fragment of presenilin with APH-1
J Biol Chem 283(50): 34677-86 -
Page RM, Baumann K, Tomioka M, Perez-Revuelta BI, Jacobsen H, Flohr A, Luebbers T, Ozmen L, Steiner H, Haass C
(2008)
Generation of Aβ38 and Aβ42 is independently and differentially affected by familial Alzheimer disease-associated presenilin mutations and γ-secretase modulation
J Biol Chem 283(2): 677-83 -
Fluhrer R, Martin L, Grammer G, Haug-Kroper M, Klier B, Winkler E, Kremmer E, Condron MM, Teplow DB, Steiner H, Haass C
(2008)
Intramembrane proteolysis of GXGD-type aspartyl proteases is slowed by a familial Alzheimer disease-like mutation
J Biol Chem 283(44): 30121-8 -
Sato T, Diehl TS, Narayanan S, Funamoto S, Ihara Y, De Strooper B, Steiner H, Haass C, Wolfe MS
(2007)
Active γ-secretase complexes contain only one of each component
J Biol Chem 282(47): 33985-93 -
Kaether C, Scheuermann J, Fassler M, Zilow S, Shirotani K, Valkova C, Novak B, Kacmar S, Steiner H, Haass C
(2007)
Endoplasmic reticulum retention of the γ-secretase complex component Pen2 by Rer1
EMBO Rep 8(8): 743-8 -
Narlawar R, Perez Revuelta BI, Baumann K, Schubenel R, Haass C, Steiner H, Schmidt B
(2007)
N-Substituted carbazolyloxyacetic acids modulate Alzheimer associated γ-secretase
Bioorg Med Chem Lett 17(1): 176-82 -
Shirotani K, Tomioka M, Kremmer E, Haass C, Steiner H
(2007)
Pathological activity of familial Alzheimer's disease-associated mutant presenilin can be executed by six different γ-secretase complexes
Neurobiol Dis 27(1): 102-7 -
Steiner H
(2007)
Sheddases and intramembrane-cleaving proteases: RIPpers of the membrane. Symposium on regulated intramembrane proteolysis
EMBO Rep 8(6): 537-41 -
Steiner H, Than M, Bode W, Haass C
(2006)
Pore-forming scissors? A first structural glimpse of γ-secretase
Trends Biochem Sci 31(9): 491-3 -
Narlawar R, Perez Revuelta BI, Haass C, Steiner H, Schmidt B, Baumann K
(2006)
Scaffold of the cyclooxygenase-2 (COX-2) inhibitor carprofen provides Alzheimer γ-secretase modulators
J Med Chem 49(26): 7588-91 -
Okochi M, Jiang J, Itoh N, Kimura R, Steiner H, Haass C, Tagami S, Takeda M
(2006)
Secretion of the Notch-1 Aβ-like peptide during Notch signaling
J Biol Chem 281(12): 7890-8 -
Bohm C, Seibel NM, Henkel B, Steiner H, Haass C, Hampe W
(2006)
SorLA signaling by regulated intramembrane proteolysis
J Biol Chem 281(21): 14547-53 -
Yamasaki A, Eimer S, Okochi M, Smialowska A, Kaether C, Baumeister R, Haass C, Steiner H
(2006)
The GxGD motif of presenilin contributes to catalytic function and substrate identification of γ-secretase
J Neurosci 26(14): 3821-8 -
Krawitz P, Haffner C, Fluhrer R, Steiner H, Schmid B, Haass C
(2005)
Differential localization and identification of a critical aspartate suggest non-redundant proteolytic functions of the presenilin homologues SPPL2b and SPPL3
J Biol Chem 280(47): 39515-23 -
Prokop S, Haass C, Steiner H
(2005)
Length and overall sequence of the PEN-2 C-terminal domain determines its function in the stabilization of presenilin fragments
J Neurochem 94(1): 57-62 -
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 -
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 -
Geling A, Steiner H, Willem M, Bally-Cuif L, Haass C
(2002)
A γ-secretase inhibitor blocks Notch signaling in vivo and causes a severe neurogenic phenotype in zebrafish
EMBO Rep 3(7): 688-94 -
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 -
Okochi M, Steiner H, Tanii H, Tomita T, Tanaka T, Iwatsubo T, Kudo T, Takeda M, Haass C
(2002)
Presenilins mediate a dual intramembranous γ-secretase cleavage of Notch-1
EMBO J 21(20): 5408-16 -
Steiner H, Revesz T, Neumann M, Romig H, Grim MG, Pesold B, Kretzschmar HA, Hardy J, Holton JL, Baumeister R, Houlden H, Haass C
(2001)
A pathogenic presenilin-1 deletion causes abberrant Aβ 42 production in the absence of congophilic amyloid plaques
J Biol Chem 276(10): 7233-9 -
Steiner H, Winkler E, Shearman MS, Prywes R, Haass C
(2001)
Endoproteolysis of the ER stress transducer ATF6 in the presence of functionally inactive presenilins
Neurobiol Dis 8(4): 717-22 -
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 -
Haass C, Steiner H
(2001)
Protofibrils, the unifying toxic molecule of neurodegenerative disorders?
Nat Neurosci 4(9): 859-60 -
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 -
Yu G, Chen F, Nishimura M, Steiner H, Tandon A, Kawarai T, Arawaka S, Supala A, Song YQ, Rogaeva E, Holmes E, Zhang DM, Milman P, Fraser P, Haass C, St George-Hyslop P
(2000)
Mutation of conserved aspartates affect maturation of presenilin 1 and presenilin 2 complexes
Acta Neurol Scand Suppl 176: 6-11 -
Yu G, Chen F, Nishimura M, Steiner H, Tandon A, Kawarai T, Arawaka S, Supala A, Song YQ, Rogaeva E, Holmes E, Zhang DM, Milman P, Fraser PE, Haass C, George-Hyslop PS
(2000)
Mutation of conserved aspartates affects maturation of both aspartate mutant and endogenous presenilin 1 and presenilin 2 complexes
J Biol Chem 275(35): 27348-53 -
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 -
Steiner H, Romig H, Pesold B, Philipp U, Baader M, Citron M, Loetscher H, Jacobsen H, Haass C
(1999)
Amyloidogenic function of the Alzheimer's disease-associated presenilin 1 in the absence of endoproteolysis
Biochemistry 38(44): 14600-5 -
Steiner H, Pesold B, Haass C
(1999)
An in vivo assay for the identification of target proteases which cleave membrane-associated substrates
FEBS Lett 463(3): 245-9 -
Steiner H, Romig H, Grim MG, Philipp U, Pesold B, Citron M, Baumeister R, Haass C
(1999)
The biological and pathological function of the presenilin-1 Δexon 9 mutation is independent of its defect to undergo proteolytic processing
J Biol Chem 274(12): 7615-8 -
Steiner H, Capell A, Pesold B, Citron M, Kloetzel PM, Selkoe DJ, Romig H, Mendla K, Haass C
(1998)
Expression of Alzheimer's disease-associated presenilin-1 is controlled by proteolytic degradation and complex formation
J Biol Chem 273(48): 32322-31 -
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 -
Steiner H, Kispal G, Zollner A, Haid A, Neupert W, Lill R
(1996)
Heme binding to a conserved Cys-Pro-Val motif is crucial for the catalytic function of mitochondrial heme lyases
J Biol Chem 271(51): 32605-11 -
Kispal G, Steiner H, Court DA, Rolinski B, Lill R
(1996)
Mitochondrial and cytosolic branched-chain amino acid transaminases from yeast, homologs of the myc oncogene-regulated Eca39 protein
J Biol Chem 271(40): 24458-64 -
Court DA, Nargang FE, Steiner H, Hodges RS, Neupert W, Lill R
(1996)
Role of the intermembrane-space domain of the preprotein receptor Tom22 in protein import into mitochondria
Mol Cell Biol 16(8): 4035-42 -
Steiner H, Zollner A, Haid A, Neupert W, Lill R
(1995)
Biogenesis of mitochondrial heme lyases in yeast. Import and folding in the intermembrane space
J Biol Chem 270(39): 22842-9 -
Wieland J, Nitsche AM, Strayle J, Steiner H, Rudolph HK
(1995)
The PMR2 gene cluster encodes functionally distinct isoforms of a putative Na+ pump in the yeast plasma membrane
EMBO J 14(16): 3870-82 -
Tsaktanis T, Kremling H, Pavsic M, von Stackelberg R, Mack B, Steiner H, Vielmuth F, Spindler V, Huang Z, Jakubowski J, Stoecklein NH, Luxenburger E, Lauber K, Lenarcic B, Gires O
Cleavage and cell adhesion properties of human epithelial cell adhesion molecule (HEPCAM)
J Biol Chem. 2015 Oct 2;290(40):24574-91. -
Winkler E, Julius A, Steiner H, Langosch D
Homodimerization protects the amyloid precursor protein C99 fragment from cleavage by γ-secretase
Biochemistry. 2015 Oct 13;54(40):6149-52. -
Matthias Hachmeister, Karolina D. Bobowski, Sebastian Hogl, Bastian Dislich, Carola Eggert, Brigitte Mack, Heidi Kremling, Sannia Sarrach, Fabian Coscia, Wolfgang Zimmermann, Harald Steiner, Stefan F. Lichtenthaler, Oliver Gires
Regulated intramembrane proteolysis and degradation of murine epithelial cell adhesion molecule mEpCAM
PLoS One. 2013 Aug 29;8(8):e71836 -
Harald Steiner
Substrate recruitment of γ-secretase and mechanism of clinical presenilin mutations revealed by photoaffinity mapping
EMBO J. 2016 May 23. pii: e201694151. [Epub ahead of print] -
Dönem Avci, Shai Fuchs, Bianca Schrul, Michal Breker, Idan Frumkin, Chia-yi Chen, Martin L. Biniossek, Elisabeth Kremmer, Oliver Schilling, Harald Steiner, Maya Schuldiner, Marius K. Lemberg
The yeast ER-intramembrane protease Ypf1 refines nutrient sensing by regulating transporter abundance
Mol Cell. 2014 Dec 4;56(5):630-40.
Review
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Lichtenthaler SF, Tschirner SK, Steiner H
(2022)
Secretases in Alzheimer's disease: Novel insights into proteolysis of APP and TREM2
Curr Opin Neurobiol. 2022 Feb;72:101-110 -
Trambauer J, Fukumori A, Steiner H
(2020)
Pathogenic Aβ generation in familial Alzheimer's disease: novel mechanistic insights and therapeutic implications
Curr Opin Neurobiol. 2020 Apr;61:73-81 -
Fukumori A, Feilen LP, Steiner H
(2020)
Substrate recruitment by γ-secretase
Semin Cell Dev Biol. 2020 Sep;105:54-63 -
Fukumori A, Trambauer J, Steiner H
(2018)
Identifizierung von Substratbindestellen in der γ-Sekretase
BIOspektrum 01/2018: 34-36 -
Steiner H, Fukumori A, Tagami S, Okochi M
(2018)
Making the final cut: pathogenic amyloid-β peptide generation by γ-secretase
Cell Stress. 2018 Oct 28;2(11):292-310 -
Langosch D, Steiner H
(2017)
Substrate processing in intramembrane proteolysis by γ-secretase - the role of protein dynamics
Biol Chem. 2017 Apr 1;398(4):441-453 -
Langosch D, Scharnagl C, Steiner H, Lemberg MK
(2015)
Understanding intramembrane proteolysis: from protein dynamics to reaction kinetics
Trends Biochem Sci. 2015 Jun;40(6):318-27 -
Haass C, Steiner H
(2011)
Regulated intramembrane proteolysis--lessons from amyloid precursor protein processing
J Neurochem. 2011 Jun;117(5):779-96. doi: 10.1111/j.1471-4159.2011.07248.x. Epub 2011 Apr 14. -
Fluhrer R, Steiner H, Haass C
(2009)
Intramembrane proteolysis by signal peptidepeptidases - a comparative discussion of GxGD-type aspartyl proteases
J Biol Chem. 2009 May 22;284(21):13975-9. Epub 2009 Feb 3. -
Steiner H, Fluhrer R, Haass C
(2008)
Intramembrane proteolysis by γ-secretase
J Biol Chem 283(44): 29627-31 -
Steiner H
(2008)
The catalytic core of γ-secretase: presenilin revisited
Curr Alzheimer Res 5(2): 147-57 -
Kaether C, Haass C, Steiner H
(2006)
Assembly, trafficking and function of γ-secretase
Neurodegener Dis 3(4-5): 275-83 -
Steiner H
(2004)
Uncovering γ-secretase
Curr Alzheimer Res 1(3): 175-81 -
Haass C, Steiner H
(2002)
Alzheimer disease γ-secretase: a complex story of GxGD-type presenilin proteases
Trends Cell Biol 12(12): 556-62 -
Steiner H, Haass C
(2001)
Nuclear signaling: a common function of presenilin substrates?
J Mol Neurosci 17(2): 193-8 -
Walter J, Kaether C, Steiner H, Haass C
(2001)
The cell biology of Alzheimer's disease: uncovering the secrets of secretases
Curr Opin Neurobiol 11(5): 585-90 -
Steiner H, Haass C
(2000)
Intramembrane proteolysis by presenilins
Nat Rev Mol Cell Biol 1(3): 217-24 -
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
Book chapter / Editorial material
-
Steiner H
(2020)
Understanding intramembrane proteolysis by γ-secretase
Semin Cell Dev Biol. 2020 Sep;105:1-2 -
Steiner H
(2006)
The players on the gamma-secretase team - The author's perspective
Nature Medicine 12(7): 767-767