Product References
PIKFYVE inhibition mitigates disease in models of diverse forms of ALS.
Cell
Hung ST,Linares GR,Chang WH,Eoh Y,Krishnan G,Mendonca S,Hong S,Shi Y,Santana M,Kueth C,Macklin-Isquierdo S,Perry S,Duhaime S,Maios C,Chang J,Perez J,Couto A,Lai J,Li Y,Alworth SV,Hendricks E,Wang Y,Zlokovic BV,Dickman DK,Parker JA,Zarnescu DC,Gao FB,Ichida JK
Thu Feb 16 00:00:00 UTC 2023
Reversing lysosome-ribosome circuit dysregulation mitigates C9FTD/ALS neurodegeneration and behaviors.
Human molecular genetics
Ma L,Liang C,Wang J,Chang Q,Wang Y,Zhang W,Du Y,Sadan J,Chen JF
Thu Apr 06 00:00:00 UTC 2023
C9ORF72-derived poly-GA DPRs undergo endocytic uptake in iAstrocytes and spread to motor neurons.
Life science alliance
Marchi PM,Marrone L,Brasseur L,Coens A,Webster CP,Bousset L,Destro M,Smith EF,Walther CG,Alfred V,Marroccella R,Graves EJ,Robinson D,Shaw AC,Wan LM,Grierson AJ,Ebbens SJ,De Vos KJ,Hautbergue GM,Ferraiuolo L,Melki R,Azzouz M
Thu Sep 01 00:00:00 UTC 2022
Human ALS/FTD brain organoid slice cultures display distinct early astrocyte and targetable neuronal pathology.
Nature neuroscience
Szebényi K,Wenger LMD,Sun Y,Dunn AWE,Limegrover CA,Gibbons GM,Conci E,Paulsen O,Mierau SB,Balmus G,Lakatos A
Mon Nov 01 00:00:00 UTC 2021
SFPQ regulates the accumulation of RNA foci and dipeptide repeat proteins from the expanded repeat mutation in C9orf72.
Journal of cell science
Malnar M,Rogelj B
Fri Feb 19 00:00:00 UTC 2021
Active poly-GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model.
EMBO molecular medicine
Zhou Q,Mareljic N,Michaelsen M,Parhizkar S,Heindl S,Nuscher B,Farny D,Czuppa M,Schludi C,Graf A,Krebs S,Blum H,Feederle R,Roth S,Haass C,Arzberger T,Liesz A,Edbauer D
Fri Feb 07 00:00:00 UTC 2020
Altered MICOS Morphology and Mitochondrial Ion Homeostasis Contribute to Poly(GR) Toxicity Associated with C9-ALS/FTD.
Cell reports
Li S,Wu Z,Li Y,Tantray I,De Stefani D,Mattarei A,Krishnan G,Gao FB,Vogel H,Lu B
Tue Aug 04 00:00:00 UTC 2020
Smcr8 deficiency disrupts axonal transport-dependent lysosomal function and promotes axonal swellings and gain of toxicity in C9ALS/FTD mouse models.
Human molecular genetics
Liang C,Shao Q,Zhang W,Yang M,Chang Q,Chen R,Chen JF
Sun Dec 01 00:00:00 UTC 2019
Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins.
Neuropathology and applied neurobiology
Davidson Y,Robinson AC,Liu X,Wu D,Troakes C,Rollinson S,Masuda-Suzukake M,Suzuki G,Nonaka T,Shi J,Tian J,Hamdalla H,Ealing J,Richardson A,Jones M,Pickering-Brown S,Snowden JS,Hasegawa M,Mann DM
Fri Apr 01 00:00:00 UTC 2016
Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins.
Neuropathology and applied neurobiology
Davidson Y,Robinson AC,Liu X,Wu D,Troakes C,Rollinson S,Masuda-Suzukake M,Suzuki G,Nonaka T,Shi J,Tian J,Hamdalla H,Ealing J,Richardson A,Jones M,Pickering-Brown S,Snowden JS,Hasegawa M,Mann DM
Fri Apr 01 00:00:00 UTC 2016
Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins.
Neuropathology and applied neurobiology
Davidson Y,Robinson AC,Liu X,Wu D,Troakes C,Rollinson S,Masuda-Suzukake M,Suzuki G,Nonaka T,Shi J,Tian J,Hamdalla H,Ealing J,Richardson A,Jones M,Pickering-Brown S,Snowden JS,Hasegawa M,Mann DM
Fri Apr 01 00:00:00 UTC 2016
Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins.
Neuropathology and applied neurobiology
Davidson Y,Robinson AC,Liu X,Wu D,Troakes C,Rollinson S,Masuda-Suzukake M,Suzuki G,Nonaka T,Shi J,Tian J,Hamdalla H,Ealing J,Richardson A,Jones M,Pickering-Brown S,Snowden JS,Hasegawa M,Mann DM
Fri Apr 01 00:00:00 UTC 2016
C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal Dementia.
Stem cells (Dayton, Ohio)
Dafinca R,Scaber J,Ababneh N,Lalic T,Weir G,Christian H,Vowles J,Douglas AG,Fletcher-Jones A,Browne C,Nakanishi M,Turner MR,Wade-Martins R,Cowley SA,Talbot K
Mon Aug 01 00:00:00 UTC 2016
C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal Dementia.
Stem cells (Dayton, Ohio)
Dafinca R,Scaber J,Ababneh N,Lalic T,Weir G,Christian H,Vowles J,Douglas AG,Fletcher-Jones A,Browne C,Nakanishi M,Turner MR,Wade-Martins R,Cowley SA,Talbot K
Mon Aug 01 00:00:00 UTC 2016
Modelling C9orf72 dipeptide repeat proteins of a physiologically relevant size.
Human molecular genetics
Bennion Callister J,Ryan S,Sim J,Rollinson S,Pickering-Brown SM
Thu Dec 01 00:00:00 UTC 2016
Modelling C9orf72 dipeptide repeat proteins of a physiologically relevant size.
Human molecular genetics
Bennion Callister J,Ryan S,Sim J,Rollinson S,Pickering-Brown SM
Thu Dec 01 00:00:00 UTC 2016
Modelling C9orf72 dipeptide repeat proteins of a physiologically relevant size.
Human molecular genetics
Bennion Callister J,Ryan S,Sim J,Rollinson S,Pickering-Brown SM
Thu Dec 01 00:00:00 UTC 2016
Modelling C9orf72 dipeptide repeat proteins of a physiologically relevant size.
Human molecular genetics
Bennion Callister J,Ryan S,Sim J,Rollinson S,Pickering-Brown SM
Thu Dec 01 00:00:00 UTC 2016