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Correction of dysregulated lipid metabolism normalizes gene expression in oligodendrocytes and prolongs lifespan in female poly-GA C9orf72 mice 纠正失调的脂质代谢可正常化少突胶质细胞中的基因表达，并延长雌性poly-GA C9orf72小鼠的寿命。 Rezaei, Ali; Kocsis-Jutka, Virag; Gunes, Zeynep I.; Zeng, Qing; Kislinger, Georg; Bauernschmitt, Franz; Isilgan, Huseyin Berkcan; Parisi, Laura R.; Kaya, Tugberk; Franzenburg, Soeren; Koppenbrink, Jonas; Knogler, Julia; Arzberger, Thomas; Farny, Daniel; Nuscher, Brigitte; Katona, Eszter; Dhingra, Ashutosh; Yang, Chao; Laclair, Katherine D.; Janjic, Aleksandar; Enard, Wolfgang; Zhou, Qihui; Hagan, Nellwyn; Ofengeim, Dimitry; Beltran, Eduardo; Gokce, Ozgun; Simons, Mikael; Liebscher, Sabine; Edbauer, Dieter 分享 收藏
Myeloid cell-specific loss of NPC1 in mice recapitulates microgliosis and neurodegeneration in patients with Niemann-Pick type C disease Dinkel, Lina; Hummel, Selina; Zenatti, Valerio; Malara, Mariagiovanna; Tillmann, Yannik; Colombo, Alessio; Monasor, Laura Sebastian; Suh, Jung H.; Logan, Todd; Roth, Stefan; Paeger, Lars; Hoffelner, Patricia; Bludau, Oliver; Schmidt, Andree; Mueller, Stephan A.; Schifferer, Martina; Nuscher, Brigitte; Njavro, Jasenka Rudan; Prestel, Matthias; Bartos, Laura M.; Wind-Mark, Karin; Slemann, Luna; Hoermann, Leonie; Kunte, Sebastian T.; Gnoerich, Johannes; Lindner, Simon; Simons, Mikael; Herms, Jochen; Paquet, Dominik; Lichtenthaler, Stefan F.; Bartenstein, Peter; Franzmeier, Nicolai; Liesz, Arthur; Grosche, Antje; Bremova-Ertl, Tatiana; Catarino, Claudia; Beblo, Skadi; Bergner, Caroline; Schneider, Susanne A.; Strupp, Michael; Di Paolo, Gilbert; Brendel, Matthias; Tahirovic, Sabina 分享 收藏
Mis-localization of endogenous TDP-43 leads to ALS-like early-stage metabolic dysfunction and progressive motor deficits Hu, Yiying; Hruscha, Alexander; Pan, Chenchen; Schifferer, Martina; Schmidt, Michael K.; Nuscher, Brigitte; Giera, Martin; Kostidis, Sarantos; Burhan, Ozge; van Bebber, Frauke; Edbauer, Dieter; Arzberger, Thomas; Haass, Christian; Schmid, Bettina 分享 收藏
Peripheral expression of brain-penetrant progranulin rescues pathologies in mouse models of frontotemporal lobar degeneration Reich, Marvin; Simon, Matthew J.; Polke, Beate; Paris, Inaki; Werner, Georg; Schrader, Christian; Spieth, Lena; Davis, Sonnet S.; Robinson, Sophie; de Melo, Gabrielly Lunkes; Schlaphoff, Lennart; Buschmann, Katrin; Berghoff, Stefan; Logan, Todd; Nuscher, Brigitte; de Weerd, Lis; Edbauer, Dieter; Simons, Mikael; Suh, Jung H.; Sandmann, Thomas; Kariolis, Mihalis S.; Devos, Sarah L.; Lewcock, Joseph W.; Paquet, Dominik; Capell, Anja; Di Paolo, Gilbert; Haass, Christian 分享 收藏
Association of Neurofilament Light Chain, [18F]PI-2620 Tau-PET, TSPO-PET, and Clinical Progression in Patients With β-Amyloid-Negative CBS Palleis, Carla; Franzmeier, Nicolai; Weidinger, Endy; Bernhardt, Alexander M.; Katzdobler, Sabrina; Wall, Stephan; Ferschmann, Christian; Harris, Stefanie; Schmitt, Julia; Schuster, Sebastian; Gnoerich, Johannes; Finze, Anika; Biechele, Gloria; Lindner, Simon; Albert, Nathalie L.; Bartenstein, Peter; Sabri, Osama; Barthel, Henryk; Rupprecht, Rainer; Nuscher, Brigitte; Stephens, Andrew W.; Rauchmann, Boris-Stephan; Perneczky, Robert; Haass, Christian; Brendel, Matthias; Levin, Johannes; Hoeglinger, Guenter U. 分享 收藏
Early Neuroaxonal Damage in Neurologic Disorders Associated With GAD65 Antibodies Eisenhut, Katharina; Faber, Jennifer; Engels, Daniel; Gerhards, Ramona; Lewerenz, Jan; Doppler, Kathrin; Sommer, Claudia; Markewitz, Robert; Falk, Kim K.; Roessling, Rosa; Pruess, Harald; Finke, Carsten; Wickel, Jonathan; Geis, Christian; Ratuszny, Dominica; Pfeffer, Lena K.; Bittner, Stefan; Piepgras, Johannes; Kraft, Andrea; Klausewitz, Jaqueline; Nuscher, Brigitte; Kuempfel, Tania; Thaler, Franziska S. 分享 收藏
Targeting the glycine-rich domain of TDP-43 with antibodies prevents its aggregation in vitro and reduces neurofilament levels in vivo Riemenschneider, Henrick; Simonetti, Francesca; Sheth, Udit; Katona, Eszter; Roth, Stefan; Hutten, Saskia; Farny, Daniel; Michaelsen, Meike; Nuscher, Brigitte; Schmidt, Michael K.; Flatley, Andrew; Schepers, Aloys; da Silva, Lara Gruijs A.; Zhou, Qihui; Klopstock, Thomas; Liesz, Arthur; Arzberger, Thomas; Herms, Jochen; Feederle, Regina; Gendron, Tania F.; Dormann, Dorothee; Edbauer, Dieter 分享 收藏
Loss of TREM2 rescues hyperactivation of microglia, but not lysosomal deficits and neurotoxicity in models of progranulin deficiency 在颗粒蛋白前体缺乏症模型中，TREM2的缺失可挽救小胶质细胞的过度活化，但溶酶体缺陷和神经毒性却无法挽救。 Reifschneider, Anika; Robinson, Sophie; van Lengerich, Bettina; Gnoerich, Johannes; Logan, Todd; Heindl, Steffanie; Vogt, Miriam A.; Weidinger, Endy; Riedl, Lina; Wind, Karin; Zatcepin, Artem; Pesaemaa, Ida; Haberl, Sophie; Nuscher, Brigitte; Kleinberger, Gernot; Klimmt, Julien; Goetzl, Julia K.; Liesz, Arthur; Buerger, Katharina; Brendel, Matthias; Levin, Johannes; Diehl-Schmid, Janine; Suh, Jung; Di Paolo, Gilbert; Lewcock, Joseph W.; Monroe, Kathryn M.; Paquet, Dominik; Capell, Anja; Haass, Christian 分享 收藏
The porphyrin TMPyP4 inhibits elongation during the noncanonical translation of the FTLD/ALS-associated GGGGCC repeat in the C9orf72 gene Mori, Kohji; Gotoh, Shiho; Yamashita, Tomoko; Uozumi, Ryota; Kawabe, Yuya; Tagami, Shinji; Kamp, Frits; Nuscher, Brigitte; Edbauer, Dieter; Haass, Christian; Nagai, Yoshitaka; Ikeda, Manabu 分享 收藏
Higher CSF sTREM2 attenuates ApoE4-related risk for cognitive decline and neurodegeneration 较高的CSF sTREM2降低认知能力下降和神经变性的ApoE4-related风险 Franzmeier, Nicolai; Suarez-Calvet, M.; Frontzkowski, Lukas; Moore, Annah; Hohman, Timothy J.; Morenas-Rodriguez, Estrella; Nuscher, Brigitte; Shaw, Leslie; Trojanowski, John Q.; Dichgans, Martin; Kleinberger, Gernot; Haass, Christian; Ewers, Michael 分享 收藏
Enhancing protective microglial activities with a dual function TREM2 antibody to the stalk region Schlepckow, Kai; Monroe, Kathryn M.; Kleinberger, Gernot; Cantuti-Castelvetri, Ludovico; Parhizkar, Samira; Xia, Dan; Willem, Michael; Werner, Georg; Pettkus, Nadine; Brunner, Bettina; Suelzen, Alice; Nuscher, Brigitte; Hampel, Heike; Xiang, Xianyuan; Feederle, Regina; Tahirovic, Sabina; Park, Joshua, I; Prorok, Rachel; Mahon, Cathal; Liang, Chun-Chi; Shi, Ju; Kim, Do Jin; Sabelstrom, Hanna; Huang, Fen; Di Paolo, Gilbert; Simons, Mikael; Lewcock, Joseph W.; Haass, Christian 分享 收藏
Active poly-GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model Zhou, Qihui; Mareljic, Nikola; Michaelsen, Meike; Parhizkar, Samira; Heindl, Steffanie; Nuscher, Brigitte; Farny, Daniel; Czuppa, Mareike; Schludi, Carina; Graf, Alexander; Krebs, Stefan; Blum, Helmut; Feederle, Regina; Roth, Stefan; Haass, Christian; Arzberger, Thomas; Liesz, Arthur; Edbauer, Dieter 分享 收藏
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Inhibition of amyloid-β plaque formation by α-synuclein Bachhuber, Teresa; Katzmarski, Natalie; McCarter, Joanna F.; Loreth, Desiree; Tahirovic, Sabina; Kamp, Frits; Abou-Ajram, Claudia; Nuscher, Brigitte; Serrano-Pozo, Alberto; Mueller, Alexandra; Prinz, Marco; Steiner, Harald; Hyman, Bradley T.; Haass, Christian; Meyer-Luehmann, Melanie 分享 收藏
Generation of a novel rodent model for DYT1 dystonia Grundmann, Kathrin; Gloeckle, Nicola; Martella, Giuseppina; Sciamanna, Giuseppe; Hauser, Till-Karsten; Yu, Libo; Castaneda, Salvador; Pichler, Bernd; Fehrenbacher, Birgit; Schaller, Martin; Nuscher, Brigitte; Haass, Christian; Hettich, Jasmin; Yue, Zhenyu; Huu Phuc Nguyen; Pisani, Antonio; Riess, Olaf; Ott, Thomas 分享 收藏
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Inhibition of mitochondrial fusion by α-synuclein is rescued by PINK1, Parkin and DJ-1 PINK1，Parkin和DJ-1可以抑制 α-突触核蛋白对线粒体融合的抑制 Kamp, Frits; Exner, Nicole; Lutz, Anne Kathrin; Wender, Nora; Hegermann, Jan; Brunner, Bettina; Nuscher, Brigitte; Bartels, Tim; Giese, Armin; Beyer, Klaus; Eimer, Stefan; Winklhofer, Konstanze F.; Haass, Christian 分享 收藏
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