Targeting the glycine-rich domain of TDP-43 with antibodies prevents its aggregation in vitro and reduces neurofilament levels in vivo

Autor: Henrick Riemenschneider, Francesca Simonetti, Udit Sheth, Eszter Katona, Stefan Roth, Saskia Hutten, Daniel Farny, Meike Michaelsen, Brigitte Nuscher, Michael K. Schmidt, Andrew Flatley, Aloys Schepers, Lara A. Gruijs da Silva, Qihui Zhou, Thomas Klopstock, Arthur Liesz, Thomas Arzberger, Jochen Herms, Regina Feederle, Tania F. Gendron, Dorothee Dormann, Dieter Edbauer

Publikováno v: Acta Neuropathologica Communications, Vol 11, Iss 1, Pp 1-26 (2023)

Abstract Cytoplasmic aggregation and concomitant nuclear clearance of the RNA-binding protein TDP-43 are found in ~ 90% of cases of amyotrophic lateral sclerosis and ~ 45% of patients living with frontotemporal lobar degeneration, but no disease-modi

Externí odkaz: https://doaj.org/article/d7d0c5c3d184474fbef7b702542a9df6

Antibodies inhibit transmission and aggregation of C9orf72 poly‐GA dipeptide repeat proteins

Autor: Qihui Zhou, Carina Lehmer, Meike Michaelsen, Kohji Mori, Dominik Alterauge, Dirk Baumjohann, Martin H Schludi, Johanna Greiling, Daniel Farny, Andrew Flatley, Regina Feederle, Stephanie May, Franziska Schreiber, Thomas Arzberger, Christoph Kuhm, Thomas Klopstock, Andreas Hermann, Christian Haass, Dieter Edbauer

Publikováno v: EMBO Molecular Medicine, Vol 9, Iss 5, Pp 687-702 (2017)

Abstract Cell‐to‐cell transmission of protein aggregates is an emerging theme in neurodegenerative disease. Here, we analyze the dipeptide repeat (DPR) proteins that form neuronal inclusions in patients with hexanucleotide repeat expansion C9orf7

Externí odkaz: https://doaj.org/article/a6497801d247415ea63dace2fed0150e

Active poly‐GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model

Autor: Regina Feederle, Dieter Edbauer, Christian Haass, Qihui Zhou, Stefan Roth, Carina Schludi, Helmut Blum, Meike Michaelsen, Mareike Czuppa, Alexander Graf, Stefan Krebs, Samira Parhizkar, Thomas Arzberger, Arthur Liesz, Brigitte Nuscher, Steffanie Heindl, Daniel Farny, Nikola Mareljic

Publikováno v: EMBO Molecular Medicine, Vol 12, Iss 2, Pp n/a-n/a (2020)
EMBO Mol. Med. 12:e10919 (2020)
EMBO molecular medicine 12(2), e10919 (2020). doi:10.15252/emmm.201910919
EMBO Molecular Medicine

The C9orf72 repeat expansion is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and/or frontotemporal dementia (FTD). Non‐canonical translation of the expanded repeat results in abundant poly‐GA inclusion pathology throughout

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::75696b017d11a320e8ff35c1f6d55cc7
https://doaj.org/article/94311c357d894315a7a860a7ca6f1ed6

Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS

Autor: Martin Hrabé de Angelis, Thomas Misgeld, Katherine D. LaClair, Monika S. Brill, Manuela Neumann, Qihui Zhou, Meike Michaelsen, Aleksandar Janjic, Wolfgang Enard, Wolfgang Wurst, Daniel Farny, Benedikt Wefers, Birgit Rathkolb, Mikolaj Cygan, Dieter Edbauer, Thomas Arzberger

Publikováno v: Acta Neuropathol. 140, 121–142 (2020)
Acta neuropathologica 140(2), 121-142 (2020). doi:10.1007/s00401-020-02176-0
Acta Neuropathologica

Expansion of a (G4C2)n repeat in C9orf72 causes amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), but the link of the five repeat-encoded dipeptide repeat (DPR) proteins to neuroinflammation, TDP-43 pathology, and neurodegenerati

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c49fb2804712e9500696e36c93f162a1
https://mediatum.ub.tum.de/1578105

Antibodies inhibit transmission and aggregation of C9orf72 poly-GA dipeptide repeat proteins

Autor: Andreas Hermann, Franziska Schreiber, Qihui Zhou, Martin H. Schludi, Dominik Alterauge, Thomas Klopstock, Christoph Kuhm, Johanna Greiling, Daniel Farny, Dirk Baumjohann, Thomas Arzberger, Christian Haass, Kohji Mori, Meike Michaelsen, Andrew Flatley, Dieter Edbauer, Carina Lehmer, Stephanie May, Regina Feederle

Publikováno v: EMBO molecular medicine 9(5), 687-702 (2017). doi:10.15252/emmm.201607054
EMBO Mol. Med. 9, 687-702 (2017)

Cell‐to‐cell transmission of protein aggregates is an emerging theme in neurodegenerative disease. Here, we analyze the dipeptide repeat (DPR) proteins that form neuronal inclusions in patients with hexanucleotide repeat expansion C9orf72 , the m

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::64b78771fa94ad11173d2565495938af