ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
about
Mechanisms of FUS mutations in familial amyotrophic lateral sclerosisToward precision medicine in amyotrophic lateral sclerosisModeling ALS and FTD with iPSC-derived neuronsThe function of RNA-binding proteins at the synapse: implications for neurodegenerationCytoplasmic sequestration of FUS/TLS associated with ALS alters histone marks through loss of nuclear protein arginine methyltransferase 1.Early lethality and neuronal proteinopathy in mice expressing cytoplasm-targeted FUS that lacks the RNA recognition motif.Pathogenesis of FUS-associated ALS and FTD: insights from rodent modelsEngineering enhanced protein disaggregases for neurodegenerative diseaseOxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutationsMotor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.Genetically modified pig models for neurodegenerative disorders.Genetic Correction of SOD1 Mutant iPSCs Reveals ERK and JNK Activated AP1 as a Driver of Neurodegeneration in Amyotrophic Lateral Sclerosis.Multistep process of FUS aggregation in the cell cytoplasm involves RNA-dependent and RNA-independent mechanisms.Potentiated Hsp104 variants suppress toxicity of diverse neurodegenerative disease-linked proteins.Functions of FUS/TLS from DNA repair to stress response: implications for ALS.Activity-dependent FUS dysregulation disrupts synaptic homeostasisPur-alpha regulates cytoplasmic stress granule dynamics and ameliorates FUS toxicity.Neuroprotective Functions for the Histone Deacetylase SIRT6Self-assembled FUS binds active chromatin and regulates gene transcription.ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP.U1 snRNP is mislocalized in ALS patient fibroblasts bearing NLS mutations in FUS and is required for motor neuron outgrowth in zebrafishA fruitful endeavor: modeling ALS in the fruit flyLinking RNA Dysfunction and Neurodegeneration in Amyotrophic Lateral Sclerosis.A Systematic Analysis of Factors Localized to Damaged Chromatin Reveals PARP-Dependent Recruitment of Transcription FactorsAmelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1.Translocated in liposarcoma regulates the distribution and function of mammalian enabled, a modulator of actin dynamics.ALS mutations in TLS/FUS disrupt target gene expression.Rodent Models of Amyotrophic Lateral SclerosisALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.Activation of HIPK2 Promotes ER Stress-Mediated Neurodegeneration in Amyotrophic Lateral Sclerosis.Cyclin A2 promotes DNA repair in the brain during both development and aging.FUS/TLS acts as an aggregation-dependent modifier of polyglutamine disease model mice.Two familial ALS proteins function in prevention/repair of transcription-associated DNA damage.FUS Mislocalization and Vulnerability to DNA Damage in ALS Patients Derived hiPSCs and Aging Motoneurons.Sequestration of PRMT1 and Nd1-L mRNA into ALS-linked FUS mutant R521C-positive aggregates contributes to neurite degeneration upon oxidative stress.Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106.RNA-binding proteins as molecular links between cancer and neurodegeneration.Functional roles of alternative splicing factors in human disease.The interplay between DNA damage response and RNA processing: the unexpected role of splicing factors as gatekeepers of genome stability.Old versus New Mechanisms in the Pathogenesis of ALS.
P2860
Q26749169-7FF715DA-6604-4E5A-988D-5EA19B754ABAQ26764950-E3A984BC-67D5-416A-9634-1501B5CE6577Q26783672-78A6E8F6-89AA-4D2F-98FF-F08E7BD79C65Q26991684-53E68F45-5BC6-4812-808A-BB8CBB65E0F1Q27301265-FD0469CE-5D3B-41B3-9071-496C7825CFA8Q27334411-48F3F640-FC3A-4978-A45B-72EECF2EB389Q28067147-42DBAB59-CA9B-471D-A649-BC5F5A92E4E0Q28083809-D14BD9E9-0B84-4426-82BE-46F381272E4AQ28383058-5615B5E1-1C2D-4104-B1C0-E3617057FAC9Q30848999-E664078C-C934-416D-A335-180A457B0BBCQ31002975-EED3D46E-FD02-4CBC-8069-62595400F124Q33559744-158BD42D-E5EC-4EB0-8FF4-F5F7634E0E4CQ34155410-BA98420D-CC7E-4554-B72A-DCCD5D29A127Q34241138-7237BD94-7067-4D28-AFE8-1178CBF0779DQ34305789-F0BAA690-2ACC-4148-BA18-BFA937BDD696Q34480824-021C8221-256C-40E7-85AC-F4119529F026Q34507318-95E6CAD0-9DAE-4C63-89FA-A1EBCD003FDCQ34554358-223B5BDC-95B9-4318-BE86-6E40CB02EFDAQ34752975-F43F5184-9356-42C2-AE50-6C004877E4A5Q35114564-47A599CD-3B94-4F7C-B5F4-DE367BB817EAQ35237055-A9BF830F-74AB-481E-B452-0639642D4511Q35268719-4BE117FD-784B-4786-BA0F-947FE966D279Q35501802-4B76929D-A608-46B5-B20C-8434B16730C5Q35640249-DD892A5B-316D-4CB4-9467-B48BBB5C961FQ35796020-63DB7726-1A5E-430C-A5FA-B324545FFE75Q35929154-FE8B1875-5ECC-44E8-B974-F652630BDF70Q36032446-1DB3573D-24CB-429D-BF3F-357062E9FE8CQ36034603-32CBD487-3954-4935-A7A4-044967179575Q36548825-C3FFD829-258D-40AD-A441-F7D4D6441296Q37079670-C83C7485-2C1E-4D15-9569-80B453CB77B6Q37194248-8595F5C9-63D8-4F32-90A6-4B4E14C2C234Q37339987-8194A0DD-78DA-49AD-ABA5-825B987211AFQ37474001-DBC517F9-4B9A-47D9-AE1B-2A30F218B95DQ37531676-61CCC8B5-C6A5-413D-96F7-A6C0625B2DA9Q37589278-12A284E4-DA38-48FC-B06C-20DFD648A063Q37616732-9C90E0C0-8D3C-4B0A-B503-0B324D7274E1Q38251313-A7AF33A3-630C-438D-BC65-A4860563452FQ38335759-34F547BB-AA38-4555-914E-B14A1127B86AQ38450793-9FCC3D31-4C69-4AC9-9369-E3690450E36BQ38700722-50057121-5241-4F88-B3CB-9C38345F99FB
P2860
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on 10 February 2014
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
@en
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
@nl
type
label
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
@en
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
@nl
prefLabel
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
@en
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
@nl
P2093
P2860
P50
P921
P356
P1476
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.
@en
P2093
Amy A Tang
Anthony J Filiano
Caitlin E Carlton
Haiyan Qiu
Hansen Lui
Hejia Wang
Kin Fai Au
Li-Huei Tsai
Michael C Oldham
P2860
P304
P356
10.1172/JCI72723
P407
P577
2014-02-10T00:00:00Z