PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in C9orf72 ALS/FTD neurons

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Location: Deutsche Nationalbibliothek Frankfurt am Main

Extent: 1 Online-Ressource.

Language: Englisch

Bibliographic citation: 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

Creator: 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.

Contributor: SpringerLink (Online service)

DOI: 10.1038/s41593-024-01589-4

URN: urn:nbn:de:101:1-2406240947112.962638193396

Rights: Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.

Last update: 14.08.2025, 10:59 AM CEST

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Associated

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)

Other Objects (12)

The nuclear import receptor Kapβ2 modifies neurotoxicity mediated by poly(GR) in C9orf72-linked ALS/FTD

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

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

Roadmap for C9ORF72 in Frontotemporal Dementia and Amyotrophic Lateral Sclerosis: Report on the C9ORF72 FTD/ALS Summit

The nuclear import receptor Kapβ2 modifies neurotoxicity mediated by poly(GR) in C9orf72-linked ALS/FTD

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

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

Roadmap for C9ORF72 in Frontotemporal Dementia and Amyotrophic Lateral Sclerosis: Report on the C9ORF72 FTD/ALS Summit

The nuclear import receptor Kapβ2 modifies neurotoxicity mediated by poly(GR) in C9orf72-linked ALS/FTD

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

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

Roadmap for C9ORF72 in Frontotemporal Dementia and Amyotrophic Lateral Sclerosis: Report on the C9ORF72 FTD/ALS Summit

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PolyGR and polyPR knock-in mice reveal a conserved neuroprotective extracellular matrix signature in C9orf72 ALS/FTD neurons

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Other Objects (12)

The nuclear import receptor Kapβ2 modifies neurotoxicity mediated by poly(GR) in C9orf72-linked ALS/FTD

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

PolyGA targets the ER stress-adaptive response by impairing GRP75 function at the MAM in C9ORF72-ALS/FTD

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

Roadmap for C9ORF72 in Frontotemporal Dementia and Amyotrophic Lateral Sclerosis: Report on the C9ORF72 FTD/ALS Summit

The nuclear import receptor Kapβ2 modifies neurotoxicity mediated by poly(GR) in C9orf72-linked ALS/FTD

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

PolyGA targets the ER stress-adaptive response by impairing GRP75 function at the MAM in C9ORF72-ALS/FTD

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

Roadmap for C9ORF72 in Frontotemporal Dementia and Amyotrophic Lateral Sclerosis: Report on the C9ORF72 FTD/ALS Summit

The nuclear import receptor Kapβ2 modifies neurotoxicity mediated by poly(GR) in C9orf72-linked ALS/FTD

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

PolyGA targets the ER stress-adaptive response by impairing GRP75 function at the MAM in C9ORF72-ALS/FTD

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

Roadmap for C9ORF72 in Frontotemporal Dementia and Amyotrophic Lateral Sclerosis: Report on the C9ORF72 FTD/ALS Summit

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