Review
Biochemistry & Molecular Biology
Kurt A. Jellinger, Gregor K. Wenning, Nadia Stefanova
Summary: Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disease with a complex clinical presentation. It shares molecular similarities with Parkinson's disease but presents unique pathological features. The debate over whether it should be classified as a prion disease or its potential human transmission remains unresolved.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Clinical Neurology
Sara A. M. Holec, Jisoo Lee, Abby Oehler, Felicia K. Ooi, Daniel A. Mordes, Steven H. Olson, Stanley B. Prusiner, Amanda L. Woerman
Summary: This study demonstrates that MSA prions can transmit neurological disease to mice expressing wild-type SNCA in a sex-dependent manner. The transmission of MSA prions shows distinct biological activities compared to the transmission of WT preformed fibrils.
ACTA NEUROPATHOLOGICA
(2022)
Article
Clinical Neurology
Marc Kermorgant, Pierre-Olivier Fernagut, Wassilios G. Meissner, Dina N. Arvanitis, Du N'Guyen, Jean-Michel Senard, Anne Pavy-Le Traon
Summary: Multiple system atrophy is a rare and progressive neurodegenerative disorder characterized by autonomic failure. This study found that baroreflex sensitivity was significantly changed in PLP-alpha-syn mice and varied with age. Additionally, impaired heart rate variability was observed in 12-month-old female PLP-alpha-syn mice, indicating cardiovascular autonomic failure.
FRONTIERS IN NEUROLOGY
(2022)
Article
Clinical Neurology
Teresa Torre-Muruzabal, Anke van der Perren, Audrey Coens, Geraldine Gelders, Anna Barber Janer, Sara Camacho-Garcia, Therese Klingstedt, Peter Nilsson, Nadia Stefanova, Ronald Melki, Veerle Baekelandt, Wouter Peelaerts
Summary: This study found that the progression of multiple system atrophy is influenced by different types of alpha Syn strains. Alpha Syn strains impact disease progression through oligodendroglial, neurotoxic, and immune-related mechanisms, leading to neurodegeneration and brain atrophy. The activation of microglial cells is associated with the structural features of alpha Syn strains.
Review
Clinical Neurology
Meghana Goolla, William P. Cheshire, Owen A. Ross, Naveen Kondru
Summary: Multiple system atrophy (MSA) is a rare and progressive neurodegenerative disorder that is challenging to diagnose accurately and early. Current diagnostic methods have limitations in sensitivity and specificity, but recent advances in molecular biomarker research offer hope for improving MSA diagnosis.
FRONTIERS IN NEUROLOGY
(2023)
Article
Clinical Neurology
Ann M. Schmeichel, Elizabeth A. Coon, Joseph E. Parisi, Wolfgang Singer, Phillip A. Low, Eduardo E. Benarroch
Summary: The study found decreased numbers of GAD-immunoreactive neurons in the LPGi and adjacent VLM regions in patients with MSA, indicating a loss of GABAergic neurons which may contribute to REM sleep-related cardiovagal and possibly respiratory dysregulation. Further research is needed to confirm these findings and explore their implications.
Article
Clinical Neurology
Margaux Teil, Sandra Dovero, Mathieu Bourdenx, Marie-Laure Arotcarena, Sandrine Camus, Gregory Porras, Marie-Laure Thiolat, Ines Trigo-Damas, Celine Perier, Cristina Estrada, Nuria Garcia-Carrillo, Michele Morari, Wassilios G. Meissner, Maria Trinidad Herrero, Miquel Vila, Jose A. Obeso, Erwan Bezard, Benjamin Dehay
Summary: Synucleinopathies, including Parkinson's disease, dementia with Lewy bodies and multiple system atrophy, are characterized by the deposit of alpha-synuclein aggregates in neurons and glial cells. A study found that inoculating brain fractions containing glial cytoplasmic inclusions from multiple system atrophy patients into non-human primates resulted in neurodegeneration, oligodendrocyte loss, demyelination, neuroinflammation and alpha-synuclein pathology. These findings suggest the potential use of this experimental model for multiple system atrophy research and therapy development.
Article
Clinical Neurology
An Cheng, Ichiro Kawahata, Yifei Wang, Wenbin Jia, Haoyang Wang, Tomoki Sekimori, Yi Chen, Hiroyoshi Suzuki, Atsushi Takeda, Nadia Stefanova, David Finkelstein, Wenbo Ma, Min Chen, Takuya Sasaki, Kohji Fukunaga
Summary: Multiple system atrophy (MSA) is a neurodegenerative disease characterized by accumulation of misfolded a-synuclein (aSyn) and myelin disruption. This study identified epsin-2 as a potential regulator of aSyn propagation in MSA brains, suggesting epsin-2 as a novel therapeutic target for MSA.
Article
Clinical Neurology
Yasuo Miki, Kunikazu Tanji, Kana Shinnai, Makoto T. Tanaka, Firat Altay, Sandrine C. Foti, Catherine Strand, Takanori Sasaki, Tomoya Kon, Shuji Shimoyama, Tomonori Furukawa, Haruo Nishijima, Hiromi Yamazaki, Yasmine T. Asi, Conceicao Bettencourt, Zane Jaunmuktane, Mari Tada, Fumiaki Mori, Hiroki Mizukami, Masahiko Tomiyama, Hilal A. Lashuel, Tammaryn Lashley, Akiyoshi Kakita, Helen Ling, Andrew J. Lees, Janice L. Holton, Thomas T. Warner, Koichi Wakabayashi
Summary: This study investigated how abnormal alpha-synuclein in the hippocampus leads to memory impairment in multiple system atrophy (MSA). The results suggest that increased alpha-synuclein oligomers may be a pathological cause of memory impairment in MSA.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
(2022)
Article
Clinical Neurology
Takashi Ando, Yuichi Riku, Akio Akagi, Hiroaki Miyahara, Mitsuaki Hirano, Toshimasa Ikeda, Hiroyuki Yabata, Ryuichi Koizumi, Chisato Oba, Saori Morozumi, Keizo Yasui, Atsuko Goto, Taiji Katayama, Satoko Sakakibara, Ikuko Aiba, Motoko Sakai, Masaaki Konagaya, Keiko Mori, Yasuhiro Ito, Hiroyuki Yuasa, Masayo Nomura, Kristine Joyce L. Porto, Jun Mitsui, Shoji Tsuji, Maya Mimuro, Yoshio Hashizume, Masahisa Katsuno, Yasushi Iwasaki, Mari Yoshida
Summary: In this study, MSA patients with prominent hippocampal involvement showed specific demographic and clinical characteristics. The severe hippocampal pathology seen in these patients suggests a potential pathological variant of MSA characterized by neuronal alpha-synucleinopathy.
Review
Cell Biology
Jen-Hsiang T. Hsiao, Onur Tanglay, Anne A. Li, Aysha Y. G. Strobbe, Woojin Scott Kim, Glenda M. Halliday, YuHong Fu
Summary: Multiple system atrophy (MSA) is a debilitating movement disorder with unknown etiology. It presents with characteristic parkinsonism and/or cerebellar dysfunction due to deterioration in specific brain regions. The early pathological events and development mechanisms of MSA are reviewed, focusing on the involvement of oligodendrocyte lineage cells and alpha-synuclein. This understanding will guide future research in MSA.
Article
Neurosciences
Ivan Martinez-Valbuena, Naomi P. Visanji, Ain Kim, Heather H. C. Lau, Raphaella W. L. So, Sohaila Alshimemeri, Andrew Gao, Michael A. Seidman, Maria R. Luquin, Joel C. Watts, Anthony E. Lang, Gabor G. Kovacs
Summary: This study revealed significant differences in the seeding activity of alpha-synuclein in the brains of patients with Multiple System Atrophy (MSA), as well as regional variations within individual brains. These findings provide important experimental groundwork for future subclassification and rapid diagnostic assays for MSA.
TRANSLATIONAL NEURODEGENERATION
(2022)
Review
Biochemistry & Molecular Biology
Chisato Kinoshita, Noriko Kubota, Koji Aoyama
Summary: Multiple system atrophy is a rare neurodegenerative disease that is characterized by oxidative stress, glutathione deficiency, and dysregulation of miRNA. These factors are closely related to the pathology of MSA.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Neurosciences
David J. Marmion, Angela A. Rutkowski, Diptaman Chatterjee, Benjamin M. Hiller, Milton H. Werner, Erwan Bezard, Deniz Kirik, Thomas McCown, Steven J. Gray, Jeffrey H. Kordower
Summary: Multiple system atrophy (MSA) is a progressive neurodegenerative disease with unclear pathogenesis. Studies using large animal models have successfully reproduced some pathological features of the disease.
NEUROBIOLOGY OF DISEASE
(2021)
Review
Clinical Neurology
Nadia Stefanova
Summary: Multiple System Atrophy (MSA) is a rare neurodegenerative disorder with unclear etiology, rapid progression, and high mortality. It shares similar neuropathology with Parkinson's disease but has specific features. Currently, there is no efficient therapy for MSA, making the development of experimental models crucial for understanding the disease mechanisms and screening potential therapies. The PLP-α-syn mouse model is a promising model that mimics the motor and non-motor features of MSA, providing insights into the pathology.
Correction
Clinical Neurology
Sabine Eschlbock, Gregor Wenning, Alessandra Fanciulli
JOURNAL OF NEURAL TRANSMISSION
(2018)
Article
Neurosciences
Serena Venezia, Violetta Refolo, Alexia Polissidis, Leonidas Stefanis, Gregor K. Wenning, Nadia Stefanova
MOLECULAR NEURODEGENERATION
(2017)
Review
Clinical Neurology
Ryan R. Walsh, Florian Krismer, Wendy R. Galpern, Gregor K. Wenning, Phillip A. Low, Glenda Halliday, Walter J. Koroshetz, Janice Holton, Niall P. Quinn, Olivier Rascol, Leslie M. Shaw, David Eidelberg, Pam Bower, Jeffrey L. Cummings, Victor Abler, Judy Biedenharn, Gal Bitan, David J. Brooks, Patrik Brundin, Hubert Fernandez, Philip Fortier, Roy Freeman, Thomas Gasser, Art Hewitt, Guenter U. Hoeglinger, Matt J. Huentelman, Poul H. Jensen, Andreas Jeromin, Un Jung Kang, Horacio Kaufmann, Lawrence Kellerman, Vikram Khurana, Thomas Klockgether, Woojin Scott Kim, Carol Langer, Peter LeWitt, Eliezer Masliah, Wassilios Meissner, Ronald Melki, Susanne Ostrowitzki, Steven Piantadosi, Werner Poewe, David Robertson, Cyndi Roemer, Dale Schenk, Michael Schlossmacher, Jeremy D. Schmahmann, Klaus Seppi, Lily Shih, Andrew Siderowf, Glenn T. Stebbins, Nadia Stefanova, Shoji Tsuji, Sharon Sutton, Jing Zhang
Editorial Material
Clinical Neurology
Gregor Wenning, John Q. Trojanowski, Horacio Kaufmann, Thomas Wisniewski, Walter A. Rocca, Phillip A. Low
ANNALS OF NEUROLOGY
(2018)
Review
Neurosciences
S. Eschlboeck, F. Krismer, G. K. Wenning
AUTONOMIC NEUROSCIENCE-BASIC & CLINICAL
(2018)
Article
Clinical Neurology
Elisabetta Indelicato, Alessandra Fanciulli, Jean Pierre Ndayisaba, Wolfgang Nachbauer, Roberta Granata, Julia Wanschitz, Michaela Wagner, Elke R. Gizewski, Werner Poewe, Gregor K. Wenning, Sylvia Boesch
CLINICAL AUTONOMIC RESEARCH
(2018)
Review
Neurosciences
Christine Kaindlstorfer, Kurt A. Jellinger, Sabine Eschlboack, Nadia Stefanova, Guenter Weiss, Gregor K. Wenning
JOURNAL OF ALZHEIMERS DISEASE
(2018)
Article
Clinical Neurology
Morinobu Seki, Klaus Seppi, Christoph Mueller, Thomas Potrusil, Georg Goebel, Eva Reiter, Michael Nocker, Ruth Steiger, Matthias Wildauer, Elke R. Gizewski, Gregor K. Wenning, Werner Poewe, Christoph Scherfler
PARKINSONISM & RELATED DISORDERS
(2018)
Article
Clinical Neurology
Noora Tuovinen, Klaus Seppi, Francesco de Pasquale, Christoph Mueller, Michael Nocker, Michael Schocke, Elke R. Gizewski, Christian Kremser, Gregor K. Wenning, Werner Poewe, Atbin Djamshidian, Christoph Scherfler, Morinobu Seki
PARKINSONISM & RELATED DISORDERS
(2018)
Article
Clinical Neurology
Stephanie Mangesius, Anna Hussl, Florian Krismer, Philipp Mahlknecht, Eva Reiter, Susanne Tagwercher, Atbin Djamshidian, Michael Schocke, Regina Esterhammer, Gregor Wenning, Christoph Mueller, Christoph Scherfler, Elke R. Gizewski, Werner Poewe, Klaus Seppi
PARKINSONISM & RELATED DISORDERS
(2018)
Article
Clinical Neurology
Christoph Mueller, Anna Hussl, Florian Krismer, Beatrice Heim, Philipp Mahlknecht, Michael Nocker, Christoph Scherfler, Katherina Mair, Regina Esterhammer, Michael Schocke, Gregor K. Wenning, Werner Poewe, Klaus Seppi
PARKINSONISM & RELATED DISORDERS
(2018)
Article
Clinical Neurology
Ambra Stefani, Anna Heidbreder, Elisabeth Brandauer, Marc Guaita, Lisa-Marie Neier, Thomas Mitterling, Joan Santamaria, Alex Iranzo, Aleksander Videnovic, Claudia Trenkwalder, Friederike Sixel-Doering, Gregor K. Wenning, Anabel Chade, Werner Poewe, Oscar S. Gershanik, Birgit Hoegl
Article
Neurosciences
Violetta Refolo, Francesco Bez, Alexia Polissidis, Daniela Kuzdas-Wood, Edith Sturm, Martina Kamaratou, Werner Poewe, Leonidas Stefanis, M. Angela Cenci, Marina Romero-Ramos, Gregor K. Wenning, Nadia Stefanova
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2018)
Article
Clinical Neurology
Ambra Stefani, Anna Heidbreder, Elisabeth Brandauer, Marc Guaita, Lisa-Marie Neier, Thomas Mitterling, Joan Santamaria, Alex Iranzo, Aleksander Videnovic, Claudia Trenkwalder, Friederike Sixel-Doering, Gregor K. Wenning, Anabel Chade, Werner Poewe, Oscar S. Gershanik, Birgit Hoegl
Review
Clinical Neurology
Cassia Overk, Edward Rockenstein, Elvira Valera, Nadia Stefanova, Gregor Wenning, Eliezer Masliah
ACTA NEUROPATHOLOGICA
(2018)