Cargando…

ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules

Mutations in the gene encoding Fused in Sarcoma (FUS) cause amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder. FUS is a predominantly nuclear DNA- and RNA-binding protein that is involved in RNA processing. Large FUS-immunoreactive inclusions fill the perikaryon of surviving mo...

Descripción completa

Detalles Bibliográficos
Autores principales:	Vance, Caroline, Scotter, Emma L., Nishimura, Agnes L., Troakes, Claire, Mitchell, Jacqueline C., Kathe, Claudia, Urwin, Hazel, Manser, Catherine, Miller, Christopher C., Hortobágyi, Tibor, Dragunow, Mike, Rogelj, Boris, Shaw, Christopher E.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Oxford University Press 2013
Materias:	Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3674807/ https://www.ncbi.nlm.nih.gov/pubmed/23474818 http://dx.doi.org/10.1093/hmg/ddt117

Ejemplares similares

ALS-FUS pathology revisited: singleton FUS mutations and an unusual case with both a FUS and TARDBP mutation
por: King, Andrew, et al.
Publicado: (2015)

ALS-linked cytoplasmic FUS assemblies are compositionally different from physiological stress granules and sequester hnRNPA3, a novel modifier of FUS toxicity
por: An, Haiyan, et al.
Publicado: (2022)

Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion
por: Mitchell, Jacqueline C., et al.
Publicado: (2012)

Abl kinase-mediated FUS Tyr526 phosphorylation alters nucleocytoplasmic FUS localization in FTLD-FUS
por: Motaln, Helena, et al.
Publicado: (2023)

FUS is sequestered in nuclear aggregates in ALS patient fibroblasts
por: Schwartz, Jacob C., et al.
Publicado: (2014)

Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasm
por: Shelkovnikova, Tatyana A, et al.
Publicado: (2013)

Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation
por: Sanjuan-Ruiz, Inmaculada, et al.
Publicado: (2021)

Aberrant Phase Separation of FUS Leads to Lysosome Sequestering and Acidification
por: Trnka, Franziska, et al.
Publicado: (2021)

FUS inclusions disrupt RNA localization by sequestering kinesin-1 and inhibiting microtubule detyrosination
por: Yasuda, Kyota, et al.
Publicado: (2017)

Frameshift peptides alter the properties of truncated FUS proteins in ALS-FUS
por: An, Haiyan, et al.
Publicado: (2020)

Isogenic FUS-eGFP iPSC Reporter Lines Enable Quantification of FUS Stress Granule Pathology that Is Rescued by Drugs Inducing Autophagy
por: Marrone, Lara, et al.
Publicado: (2018)

Cytoplasmic TDP-43 is involved in cell fate during stress recovery
por: Lee, Youn-Bok, et al.
Publicado: (2021)

Expanded G4C2 repeats linked to C9ORF72 ALS and FTD form length-dependent RNA foci, sequester RNA binding proteins and are neurotoxic
por: Lee, Youn-Bok, et al.
Publicado: (2013)

Analysis of TDP-43 and its binding partners in neurodegenerative diseases
por: Kattuah, Wejdan, et al.
Publicado: (2013)

Hexanucleotide Repeats in ALS/FTD Form Length-Dependent RNA Foci, Sequester RNA Binding Proteins, and Are Neurotoxic
por: Lee, Youn-Bok, et al.
Publicado: (2013)

ALS-Associated FUS Mutations Result in Compromised FUS Alternative Splicing and Autoregulation
por: Zhou, Yueqin, et al.
Publicado: (2013)

The FUS gene is dual‐coding with both proteins contributing to FUS‐mediated toxicity
por: Brunet, Marie A, et al.
Publicado: (2020)

Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain
por: Rogelj, Boris, et al.
Publicado: (2012)

Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species
por: Scotter, Emma L., et al.
Publicado: (2014)

Does ALS‐FUS without FUS mutation represent ALS‐FET? Report of three cases
por: Borrego‐Écija, S., et al.
Publicado: (2018)

ALS/FTD-associated protein FUS induces mitochondrial dysfunction by preferentially sequestering respiratory chain complex mRNAs
por: Tsai, Yueh-Lin, et al.
Publicado: (2020)

Amyotrophic lateral sclerosis-linked FUS/TLS alters stress granule assembly and dynamics
por: Baron, Desiree M, et al.
Publicado: (2013)

Functional interaction between FUS and SMN underlies SMA-like splicing changes in wild-type hFUS mice
por: Mirra, Alessia, et al.
Publicado: (2017)

Regulation of RNG105/caprin1 dynamics by pathogenic cytoplasmic FUS and TDP-43 in neuronal RNA granules modulates synaptic loss
por: Horio, Tomoyo, et al.
Publicado: (2023)

Generation of six induced pluripotent stem cell lines from patients with amyotrophic lateral sclerosis with associated genetic mutations in either FUS or ANXA11
por: Hedges, Erin C., et al.
Publicado: (2021)

Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization
por: Casci, Ian, et al.
Publicado: (2019)

Immune activation and MRgFUS
por: Ferrara, Katherine, et al.
Publicado: (2015)

The FUS-DDIT3 Interactome in Myxoid Liposarcoma()
por: Yu, Jamie S.E., et al.
Publicado: (2019)

Multistep process of FUS aggregation in the cell cytoplasm involves RNA-dependent and RNA-independent mechanisms
por: Shelkovnikova, Tatyana A., et al.
Publicado: (2014)

Heterogeneous Nuclear Ribonucleoprotein E2 (hnRNP E2) Is a Component of TDP-43 Aggregates Specifically in the A and C Pathological Subtypes of Frontotemporal Lobar Degeneration
por: Kattuah, Wejdan, et al.
Publicado: (2019)

Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation
por: Naumann, Marcel, et al.
Publicado: (2018)

FUS ALS-causative mutations impair FUS autoregulation and splicing factor networks through intron retention
por: Humphrey, Jack, et al.
Publicado: (2020)

Casein kinase 1δ/ε phosphorylates fused in sarcoma (FUS) and ameliorates FUS-mediated neurodegeneration
por: Kishino, Yuya, et al.
Publicado: (2022)

Publisher Correction: Functional interaction between FUS and SMN underlies SMA-like splicing changes in wild-type hFUS mice
por: Mirra, Alessia, et al.
Publicado: (2018)

TLS/FUS: A protein in cancer and ALS
por: Tan, Adelene Y., et al.
Publicado: (2012)

The RNA-binding protein Fus directs translation of localized mRNAs in APC-RNP granules
por: Yasuda, Kyota, et al.
Publicado: (2013)

Endogenous TDP-43, but not FUS, contributes to stress granule assembly via G3BP
por: Aulas, Anaïs, et al.
Publicado: (2012)

ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS
por: López-Erauskin, Jone, et al.
Publicado: (2018)

ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS
por: López-Erauskin, Jone, et al.
Publicado: (2020)

Fus Expression Patterns in Developing Tooth
por: Kim, Eun-Jung, et al.
Publicado: (2013)

Cannot write session to /tmp/vufind_sessions/sess_ctbsko6qqcost1e70f399v3129