Cargando…

Impaired 26S Proteasome Assembly Precedes Neuronal Loss in Mutant UBQLN2 Rats

Proteasomal dysfunction is known to be associated with amyotrophic lateral sclerosis and frontotemporal degeneration (ALS/FTD). Our previous reports have shown that a mutant form of ubiquilin-2 (UBQLN2) linked to ALS/FTD leads to neurodegeneration accompanied by accumulations of the proteasome subun...

Descripción completa

Detalles Bibliográficos
Autores principales:	Zhang, Wenjuan, Huang, Bo, Gao, Limo, Huang, Cao
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2021
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8122323/ https://www.ncbi.nlm.nih.gov/pubmed/33919255 http://dx.doi.org/10.3390/ijms22094319

Ejemplares similares

Mutant UBQLN2(P497H) in motor neurons leads to ALS-like phenotypes and defective autophagy in rats
por: Chen, Tianhong, et al.
Publicado: (2018)

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome
por: Hjerpe, Roland, et al.
Publicado: (2016)

UBQLN2 Promotes the Production of Type I Interferon via the TBK1-IRF3 Pathway
por: Chen, Tianhong, et al.
Publicado: (2020)

Early Impairment of Synaptic and Intrinsic Excitability in Mice Expressing ALS/Dementia-Linked Mutant UBQLN2
por: Radzicki, Daniel, et al.
Publicado: (2016)

Short N-terminal disordered regions and the proline-rich domain are major regulators of phase transitions for full-length UBQLN1, UBQLN2 and UBQLN4
por: Dao, Thuy P., et al.
Publicado: (2023)

Overexpression of UBQLN1 reduces neuropathology in the P497S UBQLN2 mouse model of ALS/FTD
por: Wang, Shaoteng, et al.
Publicado: (2020)

UBQLN4 promotes STING proteasomal degradation during cisplatin‐induced DNA damage in triple‐negative breast cancer
por: Shoji, Yoshiaki, et al.
Publicado: (2022)

Towards a molecular understanding of the overlapping and distinct roles of UBQLN1 and UBQLN2 in lung cancer progression and metastasis
por: Shah, Parag P., et al.
Publicado: (2022)

UBQLN2 restrains the domesticated retrotransposon PEG10 to maintain neuronal health in ALS
por: Black, Holly H, et al.
Publicado: (2023)

Precise assembly and regulation of 26S proteasome and correlation between proteasome dysfunction and neurodegenerative diseases
por: Im, Eunju, et al.
Publicado: (2016)

Prognostic and predicted significance of Ubqln2 in patients with hepatocellular carcinoma
por: Luo, Yuan‐Deng, et al.
Publicado: (2020)

Dedifferentiation of Neurons Precedes Tumor Formation in lola Mutants
por: Southall, Tony D., et al.
Publicado: (2014)

Reversible phosphorylation of the 26S proteasome
por: Guo, Xing, et al.
Publicado: (2017)

Poly-Ub-Substrate-Degradative Activity of 26S Proteasome Is Not Impaired in the Aging Rat Brain
por: Giannini, Carolin, et al.
Publicado: (2013)

Global proteomics of Ubqln2-based murine models of ALS
por: Whiteley, Alexandra M., et al.
Publicado: (2020)

UBQLN Family Members Regulate MYC in Lung Adenocarcinoma Cells
por: Shah, Parag P., et al.
Publicado: (2023)

Proteasome in action: substrate degradation by the 26S proteasome
por: Sahu, Indrajit, et al.
Publicado: (2021)

Genomic Amplification of UBQLN4 Is a Prognostic and Treatment Resistance Factor
por: Kobayashi, Yuta, et al.
Publicado: (2022)

Axon guidance genes modulate neurotoxicity of ALS-associated UBQLN2
por: Kim, Sang Hwa, et al.
Publicado: (2023)

Multi-omics profiling identifies a deregulated FUS-MAP1B axis in ALS/FTD–associated UBQLN2 mutants
por: Strohm, Laura, et al.
Publicado: (2022)

No Evidence for Pathogenic Role of UBQLN2 Mutations in Sporadic Amyotrophic Lateral Sclerosis in the Mainland Chinese Population
por: Huang, Xiao, et al.
Publicado: (2017)

Knockdown of UBQLN1 Functions as a Strategy to Inhibit CRC Progression through the ERK-c-Myc Pathway
por: Ni, Ruoxuan, et al.
Publicado: (2023)

Regulation of Repair by the 26S Proteasome
por: Sweder, K., et al.
Publicado: (2002)

The Hunt for Degrons of the 26S Proteasome
por: Ella, Hadar, et al.
Publicado: (2019)

Aberrant neural activity in prefrontal pyramidal neurons lacking TDP-43 precedes neuron loss
por: Liang, Bo, et al.
Publicado: (2022)

Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia
por: Renaud, Laurence, et al.
Publicado: (2019)

Serpin neuropathology in the P497S UBQLN2 mouse model of ALS/FTD
por: Higgins, Nicole R., et al.
Publicado: (2021)

A novel ALS-associated variant in UBQLN4 regulates motor axon morphogenesis
por: Edens, Brittany M, et al.
Publicado: (2017)

UBQLN1 deficiency mediates telomere shortening and IPF through interacting with RPA1
por: Zhou, Haoxian, et al.
Publicado: (2023)

Autoregulation of the 26S proteasome by in situ ubiquitination
por: Jacobson, Andrew D., et al.
Publicado: (2014)

The 26S Proteasome and Initiation of Gene Transcription
por: Durairaj, Geetha, et al.
Publicado: (2014)

Continued 26S proteasome dysfunction in mouse brain cortical neurons impairs autophagy and the Keap1-Nrf2 oxidative defence pathway
por: Ugun-Klusek, Aslihan, et al.
Publicado: (2017)

Fission Yeast 26S Proteasome Mutants Are Multi-Drug Resistant Due to Stabilization of the Pap1 Transcription Factor
por: Penney, Mary, et al.
Publicado: (2012)

Early microgliosis precedes neuronal loss and behavioural impairment in mice with a frontotemporal dementia-causing CHMP2B mutation
por: Clayton, Emma L., et al.
Publicado: (2017)

Disease-Associated Mutant Ubiquitin Causes Proteasomal Impairment and Enhances the Toxicity of Protein Aggregates
por: Tank, Elizabeth M. H., et al.
Publicado: (2009)

The 26S proteasome drives trinucleotide repeat expansions
por: Concannon, Claire, et al.
Publicado: (2013)

Characterization of the 26S proteasome network in Plasmodium falciparum
por: Wang, Lihui, et al.
Publicado: (2015)

Oncogenic addiction to high 26S proteasome level
por: Tsvetkov, Peter, et al.
Publicado: (2018)

Transcriptional regulation of the 26S proteasome by Nrf1
por: KOIZUMI, Shun, et al.
Publicado: (2018)

Cryo-EM structure of the plant 26S proteasome
por: Kandolf, Susanne, et al.
Publicado: (2022)

Cannot write session to /tmp/vufind_sessions/sess_rl48l80jmq4b0us87sodbv965h