Zobrazeno 1 - 10
of 17
pro vyhledávání: '"metabolism [Proteasome Endopeptidase Complex]"'
Autor:
Henrick Riemenschneider, Qiang Guo, Jakob Bader, Frédéric Frottin, Daniel Farny, Gernot Kleinberger, Christian Haass, Matthias Mann, F. Ulrich Hartl, Wolfgang Baumeister, Mark S Hipp, Felix Meissner, Rubén Fernández‐Busnadiego, Dieter Edbauer
Publikováno v:
Embo Reports, 23(6):e53890. Wiley
EMBO reports 23(6), e53890 (2022). doi:10.15252/embr.202153890
EMBO Reports
EMBO Reports, 2022, 23, ⟨10.15252/embr.202153890⟩
EMBO reports 23(6), e53890 (2022). doi:10.15252/embr.202153890
EMBO Reports
EMBO Reports, 2022, 23, ⟨10.15252/embr.202153890⟩
International audience; Aggregation of the multifunctional RNA-binding protein TDP-43 defines large subgroups of amyotrophic lateral sclerosis and frontotemporal dementia and correlates with neurodegeneration in both diseases. In disease, characteris
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::68f47f8b65554198f2de3cc8266a570e
https://research.rug.nl/en/publications/0af60e3e-49be-447c-9742-e5a5283a2b78
https://research.rug.nl/en/publications/0af60e3e-49be-447c-9742-e5a5283a2b78
Autor:
Anja U. Bräuer, Maria Vittoria Cubellis, Georg Fuellen, Jan Lukas, Anne-Katrin Giese, Arndt Rolfs, Mathias Ernst, Giuseppina Andreotti, Stephan Struckmann, Andreas Hermann, Linda Rebecca Haake, Anne-Marie Knospe, Claudia Cozma, Valentina Citro, Susanne Seemann, Chiara Cimmaruta, Dirk Koczan
Publikováno v:
Biochemical journal (Online) (2020). doi:10.1042/BCJ20190513
info:cnr-pdr/source/autori:Susanne Seemann ; Mathias Ernst ; Chiara Cimmaruta ; Stephan Struckmann ; Claudia Cozma ; Dirk Koczan ; Anne-Marie Knospe ; Linda R Haake ; Valentina Citro ; Anja U Bräuer ; Giuseppina Andreotti ; Maria V Cubellis ; Georg Fuellen ; Andreas Hermann ; Anne-Katrin Giese ; Arndt Rolfs ; Jan Lukas/titolo:Proteostasis regulators modulate proteasomal activity and gene expression to attenuate multiple phenotypesin Fabry disease/doi:10.1042%2FBCJ20190513/rivista:Biochemical journal (Online)/anno:2020/pagina_da:/pagina_a:/intervallo_pagine:/volume
The biochemical journal / Reviews 477(2), 359-380 (2020). doi:10.1042/BCJ20190513
Biochemical Journal
info:cnr-pdr/source/autori:Susanne Seemann ; Mathias Ernst ; Chiara Cimmaruta ; Stephan Struckmann ; Claudia Cozma ; Dirk Koczan ; Anne-Marie Knospe ; Linda R Haake ; Valentina Citro ; Anja U Bräuer ; Giuseppina Andreotti ; Maria V Cubellis ; Georg Fuellen ; Andreas Hermann ; Anne-Katrin Giese ; Arndt Rolfs ; Jan Lukas/titolo:Proteostasis regulators modulate proteasomal activity and gene expression to attenuate multiple phenotypesin Fabry disease/doi:10.1042%2FBCJ20190513/rivista:Biochemical journal (Online)/anno:2020/pagina_da:/pagina_a:/intervallo_pagine:/volume
The biochemical journal / Reviews 477(2), 359-380 (2020). doi:10.1042/BCJ20190513
Biochemical Journal
The lysosomal storage disorder Fabry disease is characterized by a deficiency of the lysosomal enzyme α-Galactosidase A. The observation that missense variants in the encoding GLA gene often lead to structural destabilization, endoplasmic reticulum
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::dfda54713078b2d84fa25ecc480bef0a
https://doi.org/10.1042/bcj20190513
https://doi.org/10.1042/bcj20190513
Autor:
Jennifer Cable, Eilika Weber‐Ban, Tim Clausen, Kylie J. Walters, Michal Sharon, Daniel J. Finley, Yangnan Gu, John Hanna, Yue Feng, Sascha Martens, Anne Simonsen, Malene Hansen, Hong Zhang, Jonathan M. Goodwin, Alessio Reggio, Chunmei Chang, Liang Ge, Brenda A. Schulman, Raymond J. Deshaies, Ivan Dikic, J. Wade Harper, Ingrid E. Wertz, Nicolas H. Thomä, Mikołaj Słabicki, Judith Frydman, Ursula Jakob, Della C. David, Eric J. Bennett, Carolyn R. Bertozzi, Richa Sardana, Vinay V. Eapen, Serena Carra
Publikováno v:
Annals of the New York Academy of Sciences 1510(1), 79-99 (2022). doi:10.1111/nyas.14745
Targeted protein degradation is critical for proper cellular function and development. Protein degradation pathways, such as the ubiquitin proteasomes system, autophagy, and endosome-lysosome pathway, must be tightly regulated to ensure proper elimin
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::88110e83b56645cb723743deb2706a33
Autor:
Katerina E. Paleologou, Hilal A. Lashuel, Eliezer Masliah, Mohamed Bilal Fares, Markus Zweckstetter, Guowei Yin, Tiago F. Outeiro, Martial K. Mbefo, Sandra Tenreiro, Madalena Pinto, Abid Oueslati
Publikováno v:
The journal of biological chemistry 290(15), 9412-9427 (2015). doi:10.1074/jbc.M114.610774
Journal of Biological Chemistry
Journal of Biological Chemistry
Although alpha-synuclein (alpha-syn) phosphorylation has been considered as a hallmark of sporadic and familial Parkinson disease (PD), little is known about the effect of PD-linked mutations on alpha-syn phosphorylation. In this study, we investigat
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7b04aad2e56d3a093c84e0b8a74b979e
https://doi.org/10.1074/jbc.m114.610774
https://doi.org/10.1074/jbc.m114.610774
Autor:
Sybille Krauß, Eva-Christina Müller, Frank Matthes, Melanie Fuchs, Andrea Köhler, Stephanie Dorn, Rainer Schneider, Susann Schweiger, Erich E. Wanker
Publikováno v:
The journal of biological chemistry 289(46), 31805-31817 (2014). doi:10.1074/jbc.M113.541219
Sonic Hedgehog (SHH)-GLI signalling plays an important role during embryogenesis and in tumorigenesis. The survival and growth of several types of cancer depend on autonomously activated SHH-GLI signalling. A protein complex containing the ubiquitin-
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9689225db772ca0bd39e977d475f56d2
https://doi.org/10.1074/jbc.m113.541219
https://doi.org/10.1074/jbc.m113.541219
Autor:
Andrea Stutz, Erik C. Hett, Kevin G. Mark, Deborah T. Hung, Louise H. Slater, Brian G. Monks, Eicke Latz, Tomohiko Kawate
Publikováno v:
Nature chemical biology 9(6), 398-405 (2013). doi:10.1038/nchembio.1236
Formation of the inflammasome, a scaffolding complex that activates caspase-1, is important in numerous diseases. Pyroptotic cell death induced by anthrax lethal toxin (LT) is a model for inflammasome-mediated caspase-1 activation. We discovered 7-de
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4b2fa131d14c40294eb62b986ae04681
https://doi.org/10.1038/nchembio.1236
https://doi.org/10.1038/nchembio.1236
Autor:
Jochen Walter, Daniel N. Mitroi, Oleg Shupliakov, Dieter Swandulla, Gerhild van Echten-Deckert, André U. Deutschmann, Indulekha Karunakaran, Dan Ehninger, Konstantine Glebov, Michael Hans, Markus H. Gräler, Maren Raucamp, Julie D. Saba, Elena Sopova
Publikováno v:
Scientific Reports
Scientific reports 6(1), 37064 (2016). doi:10.1038/srep37064
Scientific reports 6(1), 37064 (2016). doi:10.1038/srep37064
The bioactive lipid sphingosine 1-phosphate (S1P) is a degradation product of sphingolipids that are particularly abundant in neurons. We have shown previously that neuronal S1P accumulation is toxic leading to ER-stress and an increase in intracellu
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6c064b74c087f5fc16aee13c4b454cf2
https://pubmed.ncbi.nlm.nih.gov/27883090
https://pubmed.ncbi.nlm.nih.gov/27883090
Autor:
Adrian Danek, Alessandro Matte, Carlo Zancanaro, Lucia De Franceschi, Elena Tibaldi, Francesca Zonta, Benedikt Bader, Anna Maria Brunati, Seth L. Alper, Mariarita Bertoldi, Alok K. Sharma, Andreas Hermann, Ruth H. Walker, Alexander Storch, Francesca Lupo, Angela Siciliano, Donatella Benati
Publikováno v:
Blood 128(25), 2976-2987 (2016). doi:10.1182/blood-2016-07-727321
Chorea-acanthocytosis is one of the hereditary neurodegenerative disorders known as the neuroacanthocytoses. Chorea-acanthocytosis is characterized by circulating acanthocytes deficient in chorein, a protein of unknown function. We report here for th
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::066e34dc3f3e1d101c1fb0f53a84d33d
Autor:
Murdoch, John D., Rostosky, Christine M., Gowrisankaran, Sindhuja, Arora, Amandeep S., Soukup, Sandra-Fausia, Vidal, Ramon, Capece, Vincenzo, Freytag, Siona, Fischer, Andre, Verstreken, Patrik, Bonn, Stefan, Raimundo, Nuno, Milosevic, Ira
Publikováno v:
Cell Reports, Vol 17, Iss 4, Pp 1071-1086 (2016)
Cell reports 17(4), 1071-1086 (2016). doi:10.1016/j.celrep.2016.09.058
Cell Reports
Cell reports 17(4), 1071-1086 (2016). doi:10.1016/j.celrep.2016.09.058
Cell Reports
Summary Endophilin-A, a well-characterized endocytic adaptor essential for synaptic vesicle recycling, has recently been linked to neurodegeneration. We report here that endophilin-A deficiency results in impaired movement, age-dependent ataxia, and
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::4f5b2fbe8ae6600dff8e31462cab31a7
http://resolver.sub.uni-goettingen.de/purl?gs-1/13844
http://resolver.sub.uni-goettingen.de/purl?gs-1/13844
Autor:
Isil Keskin, Elin Forsgren, Jonathan D. Gilthorpe, Anna Birve, Dale J. Lange, Peter M. Andersen, Stefan L. Marklund, Matthis Synofzik, Markus Weber
Publikováno v:
PLOS ONE 11(2), e0150133 (2016). doi:10.1371/journal.pone.0150133
PLoS ONE, Vol 11, Iss 2, p e0150133 (2016)
PLoS ONE
PLoS ONE, Vol 11, Iss 2, p e0150133 (2016)
PLoS ONE
Mutations in superoxide dismutase-1 (SOD1) are a common known cause of amyotrophic lateral sclerosis (ALS). The neurotoxicity of mutant SOD1s is most likely caused by misfolded molecular species, but disease pathogenesis is still not understood. Prop
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a04c3ab95ca3fc0c1f8e436bc49b5f91
https://pub.dzne.de/record/138409
https://pub.dzne.de/record/138409