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Search results 101 to 160 out of 160 for C9orf72

Category restricted to Publication

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Publication

35383205 | J:351855

First Author:	Piao X
Year:	2022
Journal:	Sci Rep
Title:	Dual-gRNA approach with limited off-target effect corrects C9ORF72 repeat expansion in vivo.
Volume:	12
Issue:	1
Pages:	5672

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Publication

36454749 | J:352174

First Author:	Jambeau M
Year:	2022
Journal:	Proc Natl Acad Sci U S A
Title:	Comprehensive evaluation of human-derived anti-poly-GA antibodies in cellular and animal models of C9orf72 disease.
Volume:	119
Issue:	49
Pages:	e2123487119

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Publication

31310593 | J:286152

First Author:	Shi Y
Year:	2019
Journal:	JCI Insight
Title:	Identification and therapeutic rescue of autophagosome and glutamate receptor defects in C9ORF72 and sporadic ALS neurons.
Volume:	5

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Publication

30825670 | J:352106

First Author:	Martier R
Year:	2019
Journal:	Mol Ther Nucleic Acids
Title:	Targeting RNA-Mediated Toxicity in C9orf72 ALS and/or FTD by RNAi-Based Gene Therapy.
Volume:	16

Pages:	26-37

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Publication

33378537 | J:304457

First Author:	Pattamatta A
Year:	2021
Journal:	Hum Mol Genet
Title:	Repeat length increases disease penetrance and severity in C9orf72 ALS/FTD BAC transgenic mice.
Volume:	29
Issue:	24
Pages:	3900-3918

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Publication

26637797 | J:228730

First Author:	Peters OM
Year:	2015
Journal:	Neuron
Title:	Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice.
Volume:	88
Issue:	5
Pages:	902-909

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Publication

31676125 | J:284819

First Author:	Ryan S
Year:	2019
Journal:	Neurobiol Aging
Title:	CRISPR/Cas9 does not facilitate stable expression of long C9orf72 dipeptides in mice.
Volume:	84

Pages:	235.e1-235.e8

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Publication

26979938 | J:269047

First Author:	Atanasio A
Year:	2016
Journal:	Sci Rep
Title:	C9orf72 ablation causes immune dysregulation characterized by leukocyte expansion, autoantibody production, and glomerulonephropathy in mice.
Volume:	6

Pages:	23204

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Publication

28409281 | J:324567

First Author:	Schludi MH
Year:	2017
Journal:	Acta Neuropathol
Title:	Spinal poly-GA inclusions in a C9orf72 mouse model trigger motor deficits and inflammation without neuron loss.
Volume:	134
Issue:	2
Pages:	241-254

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Publication

37845749 | J:357886

First Author:	Bennett CL
Year:	2023
Journal:	Acta Neuropathol Commun
Title:	Senataxin helicase, the causal gene defect in ALS4, is a significant modifier of C9orf72 ALS G4C2 and arginine-containing dipeptide repeat toxicity.
Volume:	11
Issue:	1
Pages:	164

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Publication

28481984 | J:243430

First Author:	Farg MA
Year:	2017
Journal:	Hum Mol Genet
Title:	The DNA damage response (DDR) is induced by the C9orf72 repeat expansion in amyotrophic lateral sclerosis.
Volume:	26
Issue:	15
Pages:	2882-2896

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Publication

31858749 | J:324627

First Author:	Zhou Q
Year:	2020
Journal:	EMBO Mol Med
Title:	Active poly-GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model.
Volume:	12
Issue:	2
Pages:	e10919

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Publication

31266945 | J:282709

First Author:	Hao Z
Year:	2019
Journal:	Nat Commun
Title:	Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR.
Volume:	10
Issue:	1
Pages:	2906

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Publication

32562018 | J:324626

First Author:	LaClair KD
Year:	2020
Journal:	Acta Neuropathol
Title:	Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS.
Volume:	140
Issue:	2
Pages:	121-142

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Publication

32727895 | J:295387

First Author:	Rosbash M
Year:	2020
Journal:	Proc Natl Acad Sci U S A
Title:	Metformin treatment of the C9orf72 ALS/FTD mouse: Almost too good for words.
Volume:	117
Issue:	33
Pages:	19627-19628

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Publication

27559131 | -

First Author:	Amick J
Year:	2016
Journal:	Mol Biol Cell
Title:	C9orf72 binds SMCR8, localizes to lysosomes, and regulates mTORC1 signaling.
Volume:	27
Issue:	20
Pages:	3040-3051

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Publication

28072389 | J:241305

First Author:	Celona B
Year:	2017
Journal:	Elife
Title:	Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106.
Volume:	6

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Publication

30765536 | J:272930

First Author:	Zhang YJ
Year:	2019
Journal:	Science
Title:	Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity.
Volume:	363
Issue:	6428

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Publication

34233951 | J:322451

First Author:	Rodriguez S
Year:	2021
Journal:	Sci Transl Med
Title:	Genome-encoded cytoplasmic double-stranded RNAs, found in C9ORF72 ALS-FTD brain, propagate neuronal loss.
Volume:	13
Issue:	601

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Publication

37319115 | -

First Author:	Yang F
Year:	2023
Journal:	J Neurochem
Title:	C9orf72 poly-PR helps p53 escape from the ubiquitin-proteasome system and promotes its stability.
Volume:	166
Issue:	2
Pages:	389-402

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Publication

28550099 | J:243647

First Author:	Herranz-Martin S
Year:	2017
Journal:	Dis Model Mech
Title:	Viral delivery of C9orf72 hexanucleotide repeat expansions in mice leads to repeat-length-dependent neuropathology and behavioural deficits.
Volume:	10
Issue:	7
Pages:	859-868

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Publication

30150298 | J:267977

First Author:	Fomin V
Year:	2018
Journal:	Mol Cell Biol
Title:	The C9ORF72 Gene, Implicated in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia, Encodes a Protein That Functions in Control of Endothelin and Glutamate Signaling.
Volume:	38
Issue:	22

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Publication

33431483 | J:304437

First Author:	Riemslagh FW
Year:	2021
Journal:	Dis Model Mech
Title:	Inducible expression of human C9ORF72 36x G₄C₂ hexanucleotide repeats is sufficient to cause RAN translation and rapid muscular atrophy in mice.

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Publication

33558503 | J:304542

First Author:	Liu Y
Year:	2021
Journal:	Nat Commun
Title:	Variant-selective stereopure oligonucleotides protect against pathologies associated with C9orf72-repeat expansion in preclinical models.
Volume:	12
Issue:	1
Pages:	847

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Publication

35106589 | J:359512

First Author:	Kandasamy P
Year:	2022
Journal:	Nucleic Acids Res
Title:	Impact of guanidine-containing backbone linkages on stereopure antisense oligonucleotides in the CNS.
Volume:	50
Issue:	10
Pages:	5401-5423

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Publication

35592494 | J:352104

First Author:	Liu Y
Year:	2022
Journal:	Mol Ther Nucleic Acids
Title:	Preclinical evaluation of WVE-004, aninvestigational stereopure oligonucleotide forthe treatment of C9orf72-associated ALS or FTD.
Volume:	28

Pages:	558-570

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Publication

38556190 | J:347657

First Author:	Fu X
Year:	2024
Journal:	Exp Neurol
Title:	DDX3X overexpression decreases dipeptide repeat proteins in a mouse model of C9ORF72-ALS/FTD.
Volume:	376

Pages:	114768

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Publication

36857431 | J:335414

First Author:	Castelli LM
Year:	2023
Journal:	Sci Transl Med
Title:	A cell-penetrant peptide blocking C9ORF72-repeat RNA nuclear export reduces the neurotoxic effects of dipeptide repeat proteins.
Volume:	15
Issue:	685
Pages:	eabo3823

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Publication

34618203 | J:357640

First Author:	Diaz-Garcia S
Year:	2021
Journal:	Acta Neuropathol
Title:	Nuclear depletion of RNA-binding protein ELAVL3 (HuC) in sporadic and familial amyotrophic lateral sclerosis.
Volume:	142
Issue:	6
Pages:	985-1001

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Publication

36409902 | J:351846

First Author:	Bush JA
Year:	2022
Journal:	Proc Natl Acad Sci U S A
Title:	A blood-brain penetrant RNA-targeted small molecule triggers elimination of r(G(4)C(2))(exp) in c9ALS/FTD via the nuclear RNA exosome.
Volume:	119
Issue:	48
Pages:	e2210532119

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Publication

34857917 | J:357709

First Author:	Wang T
Year:	2022
Journal:	Cell Death Differ
Title:	Ferroptosis mediates selective motor neuron death in amyotrophic lateral sclerosis.
Volume:	29
Issue:	6
Pages:	1187-1198

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Publication

23608112 | J:211734

First Author:	Herdewyn S
Year:	2013
Journal:	Neurobiol Aging
Title:	Progranulin does not affect motor neuron degeneration in mutant SOD1 mice and rats.
Volume:	34
Issue:	10
Pages:	2302-3

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Publication

29415125 | J:258732

First Author:	Bento-Abreu A
Year:	2018
Journal:	Hum Mol Genet
Title:	Elongator subunit 3 (ELP3) modifies ALS through tRNA modification.
Volume:	27
Issue:	7
Pages:	1276-1289

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Publication

30310141 | J:268073

First Author:	Porta S
Year:	2018
Journal:	Nat Commun
Title:	Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo.
Volume:	9
Issue:	1
Pages:	4220

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Publication

31061493 | J:281537

First Author:	Lu J
Year:	2019
Journal:	Nat Neurosci
Title:	L3MBTL1 regulates ALS/FTD-associated proteotoxicity and quality control.
Volume:	22
Issue:	6
Pages:	875-886

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Publication

27796305 | J:242632

First Author:	Tian F
Year:	2016
Journal:	Nat Commun
Title:	Monitoring peripheral nerve degeneration in ALS by label-free stimulated Raman scattering imaging.
Volume:	7

Pages:	13283

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Publication

35697676 | J:326744

First Author:	Gleixner AM
Year:	2022
Journal:	Nat Commun
Title:	NUP62 localizes to ALS/FTLD pathological assemblies and contributes to TDP-43 insolubility.
Volume:	13
Issue:	1
Pages:	3380

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Publication

27321923 | J:239629

First Author:	Lee S
Year:	2016
Journal:	Neuron
Title:	Activation of HIPK2 Promotes ER Stress-Mediated Neurodegeneration in Amyotrophic Lateral Sclerosis.
Volume:	91
Issue:	1
Pages:	41-55

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Publication

28093491 | J:241667

First Author:	Clayton EL
Year:	2017
Journal:	Hum Mol Genet
Title:	Early microgliosis precedes neuronal loss and behavioural impairment in mice with a frontotemporal dementia-causing CHMP2B mutation.
Volume:	26
Issue:	5
Pages:	873-887

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Publication

28978466 | J:254206

First Author:	Coyne AN
Year:	2017
Journal:	Cell Rep
Title:	Post-transcriptional Inhibition of Hsc70-4/HSPA8 Expression Leads to Synaptic Vesicle Cycling Defects in Multiple Models of ALS.
Volume:	21
Issue:	1
Pages:	110-125

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Publication

35379876 | J:338779

First Author:	Verdone BM
Year:	2022
Journal:	Sci Rep
Title:	A mouse model with widespread expression of the C9orf72-linked glycine-arginine dipeptide displays non-lethal ALS/FTD-like phenotypes.
Volume:	12
Issue:	1
Pages:	5644

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Publication

29458840 | J:262424

First Author:	Matamala JM
Year:	2018
Journal:	Neurobiol Aging
Title:	Genome-wide circulating microRNA expression profiling reveals potential biomarkers for amyotrophic lateral sclerosis.
Volume:	64

Pages:	123-138

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Publication

34239068 | J:357510

First Author:	Luecke IW
Year:	2023
Journal:	Gene Ther
Title:	Viral vector gene delivery of the novel chaperone protein SRCP1 to modify insoluble protein in in vitro and in vivo models of ALS.
Volume:	30
Issue:	6
Pages:	528-533

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Publication

31158439 | J:282904

First Author:	Van Acker ZP
Year:	2019
Journal:	Neuroscience
Title:	Non-Methylation-Linked Mechanism of REST-Induced Neuroglobin Expression Impacts Mitochondrial Phenotypes in a Mouse Model of Amyotrophic Lateral Sclerosis.
Volume:	412

Pages:	233-247

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Publication

28539470 | J:252170

First Author:	Imamura K
Year:	2017
Journal:	Sci Transl Med
Title:	The Src/c-Abl pathway is a potential therapeutic target in amyotrophic lateral sclerosis.
Volume:	9
Issue:	391

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Publication

28344074 | J:244047

First Author:	Shang J
Year:	2017
Journal:	Neuroscience
Title:	Aberrant distributions of nuclear pore complex proteins in ALS mice and ALS patients.
Volume:	350

Pages:	158-168

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Publication

24667415 | J:213426

First Author:	Bros-Facer V
Year:	2014
Journal:	Hum Mol Genet
Title:	Treatment with an antibody directed against Nogo-A delays disease progression in the SOD1G93A mouse model of Amyotrophic lateral sclerosis.
Volume:	23
Issue:	16
Pages:	4187-200

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Publication

30442666 | J:268243

First Author:	McAlpine W
Year:	2018
Journal:	Proc Natl Acad Sci U S A
Title:	Excessive endosomal TLR signaling causes inflammatory disease in mice with defective SMCR8-WDR41-C9ORF72 complex function.
Volume:	115
Issue:	49
Pages:	E11523-E11531

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Publication

30496365 | J:303238

First Author:	Clayton EL
Year:	2018
Journal:	Brain
Title:	Frontotemporal dementia causative CHMP2B impairs neuronal endolysosomal traffic-rescue by TMEM106B knockdown.
Volume:	141
Issue:	12
Pages:	3428-3442

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Publication

32415281 | J:302747

First Author:	Gandelman M
Year:	2020
Journal:	Cell Death Differ
Title:	Staufen 1 amplifies proapoptotic activation of the unfolded protein response.
Volume:	27
Issue:	10
Pages:	2942-2951

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Publication

27126638 | J:236991

First Author:	Busch JI
Year:	2016
Journal:	Hum Mol Genet
Title:	Increased expression of the frontotemporal dementia risk factor TMEM106B causes C9orf72-dependent alterations in lysosomes.
Volume:	25
Issue:	13
Pages:	2681-2697

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Publication

25193032 | J:253702

First Author:	Ajroud-Driss S
Year:	2015
Journal:	Biochim Biophys Acta
Title:	Sporadic and hereditary amyotrophic lateral sclerosis (ALS).
Volume:	1852
Issue:	4
Pages:	679-84

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Publication

32347002 | J:324634

First Author:	Jensen BK
Year:	2020
Journal:	EMBO Mol Med
Title:	Synaptic dysfunction induced by glycine-alanine dipeptides in C9orf72-ALS/FTD is rescued by SV2 replenishment.
Volume:	12
Issue:	5
Pages:	e10722

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Publication

33028409 | J:308483

First Author:	Huang M
Year:	2020
Journal:	Acta Neuropathol Commun
Title:	Network analysis of the progranulin-deficient mouse brain proteome reveals pathogenic mechanisms shared in human frontotemporal dementia caused by GRN mutations.
Volume:	8
Issue:	1
Pages:	163

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Publication

29855382 | J:263969

First Author:	Nicholson AM
Year:	2018
Journal:	Acta Neuropathol Commun
Title:	Loss of Tmem106b is unable to ameliorate frontotemporal dementia-like phenotypes in an AAV mouse model of C9ORF72-repeat induced toxicity.
Volume:	6
Issue:	1
Pages:	42

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Publication

34118929 | J:314497

First Author:	Milani M
Year:	2021
Journal:	J Neuroinflammation
Title:	Targeting S100A4 with niclosamide attenuates inflammatory and profibrotic pathways in models of amyotrophic lateral sclerosis.
Volume:	18
Issue:	1
Pages:	132

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Publication

35507432 | J:344849

First Author:	Wang SM
Year:	2023
Journal:	Autophagy
Title:	Nucleoporin POM121 signals TFEB-mediated autophagy via activation of SIGMAR1/sigma-1 receptor chaperone by pridopidine.
Volume:	19
Issue:	1
Pages:	126-151

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Publication

35276083 | J:326153

First Author:	Arredondo C
Year:	2022
Journal:	Neuron
Title:	Excessive release of inorganic polyphosphate by ALS/FTD astrocytes causes non-cell-autonomous toxicity to motoneurons.
Volume:	110
Issue:	10
Pages:	1656-1670.e12

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Publication

15489334 | -

First Author:	Gerhard DS
Year:	2004
Journal:	Genome Res
Title:	The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Volume:	14
Issue:	10B
Pages:	2121-7

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Publication

19468303 | -

First Author:	Church DM
Year:	2009
Journal:	PLoS Biol
Title:	Lineage-specific biology revealed by a finished genome assembly of the mouse.
Volume:	7
Issue:	5
Pages:	e1000112

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Search results 101 to 160 out of 160 for C9orf72

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