Výsledky vyhledávání - "frontotemporal lobar degeneration (FTLD)"

Akademický článek

Frontotemporal dementia-like disease progression elicited by seeded aggregation and spread of FUS

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

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Akademický článek

Increase in wasteosomes (corpora amylacea) in frontotemporal lobar degeneration with specific detection of tau, TDP-43 and FUS pathology

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

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Akademický článek

Lipopolysaccharide triggers exacerbated microglial activation, excessive cytokine release and behavioural disturbances in mice with truncated Fused-in-Sarcoma Protein (FUS)

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

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Akademický článek

Feasibility and acceptability of remote smartphone cognitive testing in frontotemporal dementia research

Publikováno v: Alzheimer’s & Dementia: Diagnosis, Assessment & Disease Monitoring, Vol 15, Iss 2, Pp n/a-n/a (2023)

Abstract Introduction Remote smartphone assessments of cognition, speech/language, and motor functioning in frontotemporal dementia (FTD) could enable decentralized clinical trials and improve access to research. We studied the feasibility and accept

Externí odkaz: https://doaj.org/article/ddb1152f42564be09bd4bebba08f13f1

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Akademický článek

Differential regulation of progranulin derived granulin peptides

Autor: Tingting Zhang, Huan Du, Mariela Nunez Santos, Xiaochun Wu, Mitchell D. Pagan, Lianne Jillian Trigiani, Nozomi Nishimura, Thomas Reinheckel, Fenghua Hu

Publikováno v: Molecular Neurodegeneration, Vol 17, Iss 1, Pp 1-12 (2022)

Abstract Background Haploinsufficiency of progranulin (PGRN) is a leading cause of frontotemporal lobar degeneration (FTLD). PGRN is comprised of 7.5 granulin repeats and is processed into individual granulin peptides in the lysosome. However, very l

Externí odkaz: https://doaj.org/article/63dfd99a4a9848ad96214c257ffd37aa

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