Cargando…

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

The cytoplasmic mislocalization and aggregation of TAR DNA-binding protein-43 (TDP-43) is a common histopathological hallmark of the amyotrophic lateral sclerosis and frontotemporal dementia disease spectrum (ALS/FTD). However, the composition of aggregates and their contribution to the disease proc...

Descripción completa

Detalles Bibliográficos
Autores principales:	Chou, Ching-Chieh, Zhang, Yi, Umoh, Mfon E., Vaughan, Spencer W., Lorenzini, Ileana, Liu, Feilin, Sayegh, Melissa, Donlin-Asp, Paul G., Chen, Yu Han, Duong, Duc M., Seyfried, Nicholas T., Powers, Maureen A., Kukar, Thomas, Hales, Chadwick M., Gearing, Marla, Cairns, Nigel J., Boylan, Kevin B., Dickson, Dennis W., Rademakers, Rosa, Zhang, Yong-Jie, Petrucelli, Leonard, Sattler, Rita, Zarnescu, Daniela C., Glass, Jonathan D., Rossoll, Wilfried
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	2018
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5800968/ https://www.ncbi.nlm.nih.gov/pubmed/29311743 http://dx.doi.org/10.1038/s41593-017-0047-3

Ejemplares similares

A proteomic network approach across the ALS‐FTD disease spectrum resolves clinical phenotypes and genetic vulnerability in human brain
por: Umoh, Mfon E, et al.
Publicado: (2017)

Modelling TDP-43 proteinopathy in Drosophila uncovers shared and neuron-specific targets across ALS and FTD relevant circuits
por: Godfrey, R. Keating, et al.
Publicado: (2023)

SUMOylation Regulates TDP-43 Splicing Activity and Nucleocytoplasmic Distribution
por: Maraschi, AnnaMaria, et al.
Publicado: (2021)

Mis-spliced transcripts generate de novo proteins in TDP-43-related ALS/FTD
por: Seddighi, Sahba, et al.
Publicado: (2023)

Microglial lysosome dysfunction contributes to white matter pathology and TDP-43 proteinopathy in GRN-associated FTD
por: Wu, Yanwei, et al.
Publicado: (2021)

Traumatic injury compromises nucleocytoplasmic transport and leads to TDP-43 pathology
por: Anderson, Eric N, et al.
Publicado: (2021)

Nuclear RNA transcript levels modulate nucleocytoplasmic distribution of ALS/FTD-associated protein FUS
por: Tsai, Yueh-Lin, et al.
Publicado: (2022)

ER–mitochondria associations are regulated by the VAPB–PTPIP51 interaction and are disrupted by ALS/FTD-associated TDP-43
por: Stoica, Radu, et al.
Publicado: (2014)

TDP-43 represses cryptic exon inclusion in the FTD–ALS gene UNC13A
por: Ma, X. Rosa, et al.
Publicado: (2022)

C-terminal and full length TDP-43 specie differ according to FTLD-TDP lesion type but not genetic mutation
por: Josephs, Keith A., et al.
Publicado: (2019)

Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models
por: Lee, Pin-Tse, et al.
Publicado: (2020)

Modifiers of C9orf72 DPR toxicity implicate nucleocytoplasmic transport impairments in c9FTD/ALS
por: Jovičić, Ana, et al.
Publicado: (2015)

Epigenetic Small Molecules Rescue Nucleocytoplasmic Transport and DNA Damage Phenotypes in C9ORF72 ALS/FTD
por: Ramic, Melina, et al.
Publicado: (2021)

TDP-43 and other hnRNPs regulate cryptic exon inclusion of a key ALS/FTD risk gene, UNC13A
por: Koike, Yuka, et al.
Publicado: (2023)

Mechanisms and models of TDP-43 proteinopathies
por: Petrucelli, Leonard
Publicado: (2012)

Rodent Models of TDP-43 Proteinopathy: Investigating the Mechanisms of TDP-43-Mediated Neurodegeneration
por: Gendron, Tania F., et al.
Publicado: (2011)

Old age amyotrophic lateral sclerosis and limbic TDP‐43 pathology
por: Murakami, Aya, et al.
Publicado: (2022)

Distinct Phospho-TDP-43 brain distribution in two cases of FTD, one associated with ALS
por: Guedes, Álvaro C.B., et al.
Publicado: (2017)

Isolation and characterization of antibody fragments selective for human FTD brain derived TDP-43 variants
por: Venkataraman, Lalitha, et al.
Publicado: (2020)

Disulfide cross-linked multimers of TDP-43 and spinal motoneuron loss in a TDP-43(A315T) ALS/FTD mouse model
por: Bargsted, Leslie, et al.
Publicado: (2017)

Correction: TDP-43 and other hnRNPs regulate cryptic exon inclusion of a key ALS/FTD risk gene, UNC13A
por: Koike, Yuka, et al.
Publicado: (2023)

Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood
por: Belzil, Veronique V., et al.
Publicado: (2013)

TDP-43 cytoplasmic inclusion formation is disrupted in C9orf72-associated amyotrophic lateral sclerosis/frontotemporal lobar degeneration
por: Lee, Samuel M, et al.
Publicado: (2019)

Coexistence of perseveration and apathy in the TDP-43(Q331K) knock-in mouse model of ALS–FTD
por: Kim, Eosu, et al.
Publicado: (2020)

C9orf72 dipeptides disrupt the nucleocytoplasmic transport machinery and cause TDP-43 mislocalisation to the cytoplasm
por: Ryan, Sarah, et al.
Publicado: (2022)

Downregulation of MicroRNA-9 in iPSC-Derived Neurons of FTD/ALS Patients with TDP-43 Mutations
por: Zhang, Zhijun, et al.
Publicado: (2013)

TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD
por: White, Matthew A., et al.
Publicado: (2018)

Cytoplasmic Expression of the ALS/FTD-Related Protein TDP-43 Decreases Global Translation Both in vitro and in vivo
por: Charif, Santiago E., et al.
Publicado: (2020)

Nuclear import receptors are recruited by FG-nucleoporins to rescue hallmarks of TDP-43 proteinopathy
por: Khalil, Bilal, et al.
Publicado: (2022)

Clinical and neuropathological features of ALS/FTD with TIA1 mutations
por: Hirsch-Reinshagen, Veronica, et al.
Publicado: (2017)

Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure
por: Ding, Xuebing, et al.
Publicado: (2015)

Induction of autophagy mitigates TDP-43 pathology and translational repression of neurofilament mRNAs in mouse models of ALS/FTD
por: Kumar, Sunny, et al.
Publicado: (2021)

Sustained therapeutic benefits by transient reduction of TDP-43 using ENA-modified antisense oligonucleotides in ALS/FTD mice
por: Takeuchi, Toshihide, et al.
Publicado: (2023)

C-terminal frameshift variant of TDP-43 with pronounced aggregation-propensity causes rimmed vacuole myopathy but not ALS/FTD
por: Ervilha Pereira, Pedro, et al.
Publicado: (2023)

TDP-1/TDP-43 Regulates Stress Signaling and Age-Dependent Proteotoxicity in Caenorhabditis elegans
por: Vaccaro, Alexandra, et al.
Publicado: (2012)

Endocytosis regulates TDP-43 toxicity and turnover
por: Liu, Guangbo, et al.
Publicado: (2017)

Network analysis of the progranulin-deficient mouse brain proteome reveals pathogenic mechanisms shared in human frontotemporal dementia caused by GRN mutations
por: Huang, Meixiang, et al.
Publicado: (2020)

To Be or Not To Be…Toxic—Is RNA Association With TDP-43 Complexes Deleterious or Protective in Neurodegeneration?
por: Loganathan, Suvithanandhini, et al.
Publicado: (2020)

Divergent Phenotypes in Mutant TDP-43 Transgenic Mice Highlight Potential Confounds in TDP-43 Transgenic Modeling
por: D’Alton, Simon, et al.
Publicado: (2014)

Examining the language and behavioural profile in FTD and ALS-FTD
por: Saxon, Jennifer A, et al.
Publicado: (2017)

Cannot write session to /tmp/vufind_sessions/sess_o1nh5iampkjehjiu2dkglbi80q