Search Results - "Riemenschneider, Henrick"

Gel‐like inclusions of C‐terminal fragments of TDP‐43 sequester stalled proteasomes in neurons by Riemenschneider, Henrick, Guo, Qiang, Bader, Jakob, Frottin, Frédéric, Farny, Daniel, Kleinberger, Gernot, Haass, Christian, Mann, Matthias, Hartl, F. Ulrich, Baumeister, Wolfgang, Hipp, Mark S, Meissner, Felix, Fernández‐Busnadiego, Rubén, Edbauer, Dieter

“…Aggregation of the multifunctional RNA‐binding protein TDP‐43 defines large subgroups of amyotrophic lateral sclerosis and frontotemporal dementia and…”
Get full text

A novel CHCHD10 mutation implicates a Mia40‐dependent mitochondrial import deficit in ALS by Lehmer, Carina, Schludi, Martin H, Ransom, Linnea, Greiling, Johanna, Junghänel, Michaela, Exner, Nicole, Riemenschneider, Henrick, van der Zee, Julie, Van Broeckhoven, Christine, Weydt, Patrick, Heneka, Michael T, Edbauer, Dieter

“…CHCHD10 mutations are linked to amyotrophic lateral sclerosis, but their mode of action is unclear. In a 29‐year‐old patient with rapid disease progression, we…”
Get full text

Targeting the glycine-rich domain of TDP-43 with antibodies prevents its aggregation in vitro and reduces neurofilament levels in vivo by 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, Gruijs da Silva, Lara A, Zhou, Qihui, Klopstock, Thomas, Liesz, Arthur, Arzberger, Thomas, Herms, Jochen, Feederle, Regina, Gendron, Tania F, Dormann, Dorothee, Edbauer, Dieter

“…Cytoplasmic aggregation and concomitant nuclear clearance of the RNA-binding protein TDP-43 are found in ~ 90% of cases of amyotrophic lateral sclerosis and ~…”
Get full text

Disease‐linked TDP‐43 hyperphosphorylation suppresses TDP‐43 condensation and aggregation by Gruijs da Silva, Lara A, Simonetti, Francesca, Hutten, Saskia, Riemenschneider, Henrick, Sternburg, Erin L, Pietrek, Lisa M, Gebel, Jakob, Dötsch, Volker, Edbauer, Dieter, Hummer, Gerhard, Stelzl, Lukas S, Dormann, Dorothee

“…Post‐translational modifications (PTMs) have emerged as key modulators of protein phase separation and have been linked to protein aggregation in…”
Get full text

Cell‐to‐cell transmission of C9orf72 poly‐(Gly‐Ala) triggers key features of ALS/FTD by Khosravi, Bahram, LaClair, Kathrine D, Riemenschneider, Henrick, Zhou, Qihui, Frottin, Frédéric, Mareljic, Nikola, Czuppa, Mareike, Farny, Daniel, Hartmann, Hannelore, Michaelsen, Meike, Arzberger, Thomas, Hartl, F Ulrich, Hipp, Mark S, Edbauer, Dieter

“…The C9orf72 repeat expansion causes amyotrophic lateral sclerosis and frontotemporal dementia, but the poor correlation between C9orf72 ‐specific pathology and…”
Get full text

Author Correction: Gel-like inclusions of C-terminal fragments of TDP-43 sequester stalled proteasomes in neurons by Riemenschneider, Henrick, Guo, Qiang, Bader, Jakob, Frottin, Frédéric, Farny, Daniel, Kleinberger, Gernot, Haass, Christian, Mann, Matthias, Hartl, F Ulrich, Baumeister, Wolfgang, Hipp, Mark S, Meissner, Felix, Fernández-Busnadiego, Rubén, Edbauer, Dieter

Get full text

Multi-omics profiling identifies a deregulated FUS-MAP1B axis in ALS/FTD–associated UBQLN2 mutants by Strohm, Laura, Hu, Zehan, Suk, Yongwon, Rühmkorf, Alina, Sternburg, Erin, Gattringer, Vanessa, Riemenschneider, Henrick, Berutti, Riccardo, Graf, Elisabeth, Weishaupt, Jochen H, Brill, Monika S, Harbauer, Angelika B, Dormann, Dorothee, Dengjel, Jörn, Edbauer, Dieter, Behrends, Christian

“…Ubiquilin-2 (UBQLN2) is a ubiquitin-binding protein that shuttles ubiquitinated proteins to proteasomal and autophagic degradation. UBQLN2 mutations are…”
Get full text