Zobrazeno 1 - 10
of 1 859
pro vyhledávání: '"ftld"'
Autor:
Sonia Vazquez-Sanchez, Britt Tilkin, Fatima Gasset-Rosa, Sitao Zhang, Diana Piol, Melissa McAlonis-Downes, Jonathan Artates, Noe Govea-Perez, Yana Verresen, Lin Guo, Don W. Cleveland, James Shorter, Sandrine Da Cruz
Publikováno v:
Molecular Neurodegeneration, Vol 19, Iss 1, Pp 1-15 (2024)
Abstract RNA binding proteins have emerged as central players in the mechanisms of many neurodegenerative diseases. In particular, a proteinopathy of fused in sarcoma (FUS) is present in some instances of familial Amyotrophic lateral sclerosis (ALS)
Externí odkaz:
https://doaj.org/article/95638d30dda343ee9bedd62a7544c554
Autor:
Raquel Alsina, Marta Riba, Agnès Pérez-Millan, Sergi Borrego-Écija, Iban Aldecoa, Clara Romera, Mircea Balasa, Anna Antonell, Albert Lladó, Yaroslau Compta, Jaume del Valle, Raquel Sánchez-Valle, Carme Pelegrí, Laura Molina-Porcel, Jordi Vilaplana
Publikováno v:
Acta Neuropathologica Communications, Vol 12, Iss 1, Pp 1-13 (2024)
Abstract Wasteosomes (or corpora amylacea) are polyglucosan bodies that appear in the human brain with aging and in some neurodegenerative diseases, and have been suggested to have a potential role in a nervous system cleaning mechanism. Despite prev
Externí odkaz:
https://doaj.org/article/b86c83d11cbe43639df93dc8a849e7ee
Autor:
Gregory Mathoux, Cecilia Boccalini, Aurelien Lathuliere, Max Scheffler, Giovanni B. Frisoni, Valentina Garibotto
Publikováno v:
EJNMMI Research, Vol 14, Iss 1, Pp 1-5 (2024)
Abstract Background This case report presents a patient with progressive memory loss and choreiform movements. Case presentation Neuropsychological tests indicated multi-domain amnestic mild cognitive impairment (aMCI), and neurological examination r
Externí odkaz:
https://doaj.org/article/4131d0a0e4174e6dafa4cd01c690db9c
Autor:
Hsiang-Yu Chang, I-Fan Wang
Publikováno v:
Scientific Reports, Vol 14, Iss 1, Pp 1-9 (2024)
Abstract A group of misfolded prone-to-aggregate domains in disease-causing proteins has recently been shown to adopt unique conformations that play a role in fundamental biological processes. These processes include the formation of membrane-less su
Externí odkaz:
https://doaj.org/article/6878aff99c5c428c9190efcaf38552e1
Publikováno v:
Brain Sciences, Vol 14, Iss 11, p 1132 (2024)
TDP-43 is a ubiquitous nuclear protein that plays a central role in neurodegenerative disorders collectively known as TDP-43 proteinopathies. Under physiological conditions, TDP-43 is primarily localized to the nucleus, but in its pathological form i
Externí odkaz:
https://doaj.org/article/294feb397f1f445c9446bff5e505b1ad
Autor:
Ruth S. Nelson, Erin L. Abner, Gregory A. Jicha, Frederick A. Schmitt, Jing Di, Donna M. Wilcock, Justin M. Barber, Linda J. Van Eldik, Yuriko Katsumata, David W. Fardo, Peter T. Nelson
Publikováno v:
Acta Neuropathologica Communications, Vol 11, Iss 1, Pp 1-19 (2023)
Abstract In addition to the memory disorders and global cognitive impairment that accompany neurodegenerative diseases, behavioral and psychological symptoms of dementia (BPSD) commonly impair quality of life and complicate clinical management. To in
Externí odkaz:
https://doaj.org/article/f20b21f32736429c942d8f87267f61a3
Autor:
Ryota Kobayashi, Hiroya Naruse, Shinobu Kawakatsu, Chifumi Iseki, Yuya Suzuki, Shingo Koyama, Daichi Morioka, Hiroyuki Ishiura, Jun Mitsui, Yasuyuki Ohta, Shoji Tsuji, Tatsushi Toda, Koichi Otani
Publikováno v:
BMC Neurology, Vol 22, Iss 1, Pp 1-6 (2022)
Abstract Background Variants in the valosin-containing protein (VCP) gene were identified as one of the causes for inclusion body myopathy associated with Paget disease of the bone and frontotemporal dementia (FTD). Previously identified pathogenic v
Externí odkaz:
https://doaj.org/article/5741575af9914cad999707841c3cfa3c
Autor:
Alexander Trofimov, Dmitrii Pavlov, Anand Goswami, Anna Gorlova, Kirill Chaprov, Aleksei Umriukhin, Allan Kalueff, Alexey Deykin, Klaus-Peter Lesch, Daniel Clive Anthony, Tatyana Strekalova
Publikováno v:
Brain, Behavior, & Immunity - Health, Vol 33, Iss , Pp 100686- (2023)
CNS inflammation, including microglial activation, in response to peripheral infections are known to contribute to the pathology of both familial and sporadic neurodegenerative disease. The relationship between Fused-in-Sarcoma Protein (FUS)-mediated
Externí odkaz:
https://doaj.org/article/80e012cd55704c19bdc25c3f316432fc
Publikováno v:
Frontiers in Neuroscience, Vol 17 (2023)
Accumulation of TMEM106B fibrils composed of cleaved C-terminal fragments (CTF) of transmembrane protein 106B (TMEM106B) has recently been observed in the brains of elderly subjects and individuals with neurodegenerative diseases. To date, one antibo
Externí odkaz:
https://doaj.org/article/edfdce68f8f145689f3df37e4c76a654
Autor:
Anne-Sophie Cabron, Uwe Borgmeyer, Julia Richter, Helga Peisker, Katharina Gutbrod, Peter Dörmann, Anja Capell, Markus Damme
Publikováno v:
Acta Neuropathologica Communications, Vol 11, Iss 1, Pp 1-13 (2023)
Abstract Genetic variants in TMEM106B are a common risk factor for frontotemporal lobar degeneration and the most important modifier of disease risk in patients with progranulin (GRN) mutations (FTLD-GRN). TMEM106B is encoding a lysosomal transmembra
Externí odkaz:
https://doaj.org/article/16fa8d32c9a846b2901774631f5e6e59