PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in C9orf72 ALS/FTD neurons
- Standort
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Deutsche Nationalbibliothek Frankfurt am Main
- Umfang
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1 Online-Ressource.
- Sprache
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Englisch
- Erschienen in
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PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in C9orf72 ALS/FTD neurons ; volume:27 ; number:4 ; day:29 ; month:2 ; year:2024 ; pages:643-655 ; date:4.2024
Nature neuroscience ; 27, Heft 4 (29.2.2024), 643-655, 4.2024
- Urheber
-
Milioto, Carmelo
Carcolé, Mireia
Giblin, Ashling
Coneys, Rachel
Attrebi, Olivia
Ahmed, Mhoriam
Harris, Samuel S.
Lee, Byung Il
Yang, Mengke
Ellingford, Robert A.
Nirujogi, Raja S.
Biggs, Daniel
Salomonsson, Sally
Zanovello, Matteo
Oliveira, Paula de
Katona, Eszter
Glaria, Idoia
Mikheenko, Alla
Geary, Bethany
Udine, Evan
Vaizoglu, Deniz
Anoar, Sharifah
Jotangiya, Khrisha
Crowley, Gerard
Smeeth, Demelza M.
Adams, Mirjam L.
Niccoli, Teresa
Rademakers, Rosa
Blitterswijk, Marka van
Devoy, Anny
Hong, Soyon
Partridge, Linda
Coyne, Alyssa N.
Fratta, Pietro
Alessi, Dario
Davies, Ben
Busche, Marc Aurel
Greensmith, Linda
Fisher, Elizabeth M.
Isaacs, Adrian M.
- Beteiligte Personen und Organisationen
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SpringerLink (Online service)
- DOI
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10.1038/s41593-024-01589-4
- URN
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urn:nbn:de:101:1-2406240947112.962638193396
- Rechteinformation
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Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
- Letzte Aktualisierung
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14.08.2025, 10:59 MESZ
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Beteiligte
- Milioto, Carmelo
- Carcolé, Mireia
- Giblin, Ashling
- Coneys, Rachel
- Attrebi, Olivia
- Ahmed, Mhoriam
- Harris, Samuel S.
- Lee, Byung Il
- Yang, Mengke
- Ellingford, Robert A.
- Nirujogi, Raja S.
- Biggs, Daniel
- Salomonsson, Sally
- Zanovello, Matteo
- Oliveira, Paula de
- Katona, Eszter
- Glaria, Idoia
- Mikheenko, Alla
- Geary, Bethany
- Udine, Evan
- Vaizoglu, Deniz
- Anoar, Sharifah
- Jotangiya, Khrisha
- Crowley, Gerard
- Smeeth, Demelza M.
- Adams, Mirjam L.
- Niccoli, Teresa
- Rademakers, Rosa
- Blitterswijk, Marka van
- Devoy, Anny
- Hong, Soyon
- Partridge, Linda
- Coyne, Alyssa N.
- Fratta, Pietro
- Alessi, Dario
- Davies, Ben
- Busche, Marc Aurel
- Greensmith, Linda
- Fisher, Elizabeth M.
- Isaacs, Adrian M.
- SpringerLink (Online service)
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Antibodies inhibit transmission and aggregation of C9orf72 poly‐ GA dipeptide repeat proteins
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Symmetric dimethylation of poly-GR correlates with disease duration in C9orf72 FTLD and ALS and reduces poly-GR phase separation and toxicity
FTD/ALS-associated poly(GR) protein impairs the Notch pathway and is recruited by poly(GA) into cytoplasmic inclusions
Cell‐to‐cell transmission of C9orf72 poly‐(Gly‐Ala) triggers key features of ALS/FTD
Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease
Poly-GA triggers TDP-43 pathology by inhibiting the proteasome and nucleocytoplasmic import in C9orf72 ALS/FTD
Poly‐ GP in cerebrospinal fluid links C9orf72 ‐associated dipeptide repeat expression to the asymptomatic phase of ALS/FTD
PolyGA targets the ER stress-adaptive response by impairing GRP75 function at the MAM in C9ORF72-ALS/FTD
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Soluble and insoluble dipeptide repeat protein measurements in C9orf72-frontotemporal dementia brains show regional differential solubility and correlation of poly-GR with clinical severity
A familial FTD associated with C9orf72 repeat expansion and dysplastic gangliocytoma