Dysfunction in nonsense-mediated decay, protein homeostasis, mitochondrial function, and brain connectivity in ALS-FUS mice with cognitive deficits

Dysfunction in nonsense-mediated decay, protein homeostasis, mitochondrial function, and brain connectivity in ALS-FUS mice with cognitive deficits

10.1186/s40478-020-01111-4

Saved in:

Bibliographic Details
Main Authors:	Ho, Wan Yun, Agrawal, Ira, Tyan, Sheue-Houy, Sanford, Emma, Chang, Wei-Tang, Lim, Kenneth, Ong, Jolynn, Tan, Bernice Siu Yan, Moe, Aung Aung Kywe, Yu, Regina, Wong, Peiyan, Tucker-Kellogg, Greg, Koo, Edward, Chuang, Kai-Hsiang, Ling, Shuo-Chien
Other Authors:	PHYSIOLOGY
Format:	Article
Published:	BioMed Central Ltd 2022
Subjects:	Amyotrophic lateral sclerosis (ALS) Auto-regulation Brain connectivity Frontotemporal dementia (FTD) Functional magnetic resonance imaging (fMRI) FUS (fused in sarcoma) Nonsense-mediated decay (NMD) Oxidation phosphorylation (OXPHOS) Protein homeostasis
Online Access:	https://scholarbank.nus.edu.sg/handle/10635/232362
Tags:	Add Tag No Tags, Be the first to tag this record!
Institution:	National University of Singapore

Similar Items

Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis
by: Ling, S.-C., et al.
Published: (2021)

MOLECULAR PATHOGENESIS STUDY OF AMYOTROPHIC LATERAL SCLEROSIS (ALS) USING FUSED IN SARCOMA (FUS) PROTEIN
by: LU YIMEI
Published: (2018)

Nonsense mediated decay in Beta-Thalassemia
by: LOO POOI ENG
Published: (2011)

MOLECULAR PATHOGENESIS STUDY OF AMYOTROPHIC LATERAL SCLEROSIS (ALS) USING ALS-RELATED PROTEINS PROFILIN-1 (PFN1) AND FUSED IN SARCOMA (FUS)
by: KANG JIAN
Published: (2021)

Deregulated expression of a longevity gene, Klotho, in the C9orf72 deletion mice with impaired synaptic plasticity and adult hippocampal neurogenesis
by: Ho, Wan Yun, et al.
Published: (2021)

UNDERSTANDING THE INFLUENCE OF TDP-43 LOSS-OF-FUNCTION ON NEUROTROPHIN SIGNALLING
by: TANN YONG YOU, JASON
Published: (2018)

Proinflammatory cytokines suppress nonsense-mediated RNA decay to impair regulated transcript isoform processing in pancreatic β cells
by: Ghiasi, Seyed M., et al.
Published: (2024)

The ALS-FTD-linked gene product, C9orf72, regulates neuronal morphogenesis via autophagy
by: HO, WAN YUN, et al.
Published: (2019)

RRM domain of ALS/FTD-causing FUS characteristic of irreversible unfolding spontaneously self-assembles into amyloid fibrils /631/1647/2258/878/1263 /631/535/878/1263 /101 /101/6 article
by: Lu, Y, et al.
Published: (2020)

Deregulated expression of a longevity gene, Klotho, in the C9orf72 deletion mice with impaired synaptic plasticity and adult hippocampal neurogenesis
by: Ho, W.Y., et al.
Published: (2021)

Emerging functions of alternative splicing coupled with nonsense-mediated decay
by: Hamid, Fursham M., et al.
Published: (2015)

Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice
by: Shelkovnikova, T.A, et al.
Published: (2020)

C9orf72 expansions are the most common cause of genetic frontotemporal dementia in a Southeast Asian cohort
by: Tan, Yi Jayne, et al.
Published: (2023)

Morphological multiparameter filtration and persistent homology in mitochondrial image analysis
by: Chung, Yu-Min, et al.
Published: (2024)

Spinal cord organoids add an extra dimension to traditional motor neuron cultures
by: Winanto, et al.
Published: (2019)

Synaptic paths to neurodegeneration: The emerging role of TDP-43 and FUS in synaptic functions
by: Ling, S.-C
Published: (2020)

BCI-controlled smart wheelchair for amyotrophic lateral sclerosis patients
by: Espiritu, Noelle Marie D., et al.
Published: (2019)

Nogo-A and Nogo-66 receptor in amyotrophic lateral sclerosis
by: Teng, F.Y.H., et al.
Published: (2011)

Distinct network topology in Alzheimer’s disease and behavioral variant frontotemporal dementia
by: Ng, Adeline Su Lyn, et al.
Published: (2022)

Proteasomal inhibition causes the formation of protein aggregates containing a wide range of proteins, including nitrated proteins
by: Hyun, D.-H., et al.
Published: (2013)

Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses
by: Kapeli, K, et al.
Published: (2020)

FUS MODULATES RUNX1-MEDIATED TRANSCRIPTION VIA PHASE SEPARATION TO MAINTAIN CELLULAR INTEGRITY IN HEMATOPOIETIC STEM CELLS AND LYMPHOID PROGENITORS
by: BIBEK DUTTA
Published: (2022)

METABOLIC PROFILING OF MOTOR NEURONS DERIVED FROM PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
by: HOR JIN HUI
Published: (2020)

Structure Function Studies of Vesicle-associated membrane protein-associated protein B (VAPB) associated with Amyotrophic lateral Sclerosis (ALS)
by: LUA SHIXIONG
Published: (2012)

Deciphering lipid dysregulation in ALS: from mechanisms to translational medicine
by: Agrawal, I, et al.
Published: (2023)

ALS-causing P56S mutation and splicing variation on the hVAPB MSP domain transform its β-sandwich fold into lipid-interacting helical conformations
by: Qin, H., et al.
Published: (2014)

Molecular diagnosis and mutation characterization in thalassemias
by: WANG WEN
Published: (2010)

Genetic mutations in RNA-binding proteins and their roles in ALS
by: Kapeli, K, et al.
Published: (2020)

Structural and functional analysis of critical proteins involved in mRNA decay
by: CHENG ZHIHONG
Published: (2010)

Targeting STAT3 and oxidative phosphorylation in oncogene-addicted tumors
by: Lee M., et al.
Published: (2020)

Patient-Derived iPSCs and iNs-Shedding New Light on the Cellular Etiology of Neurodegenerative Diseases
by: Tang, Bor Luen
Published: (2023)

Nogo signaling and non-physical injury-induced nervous system pathology
by: Teng, F.Y.H., et al.
Published: (2013)

Clinical utility of neuroprotective agents in neurodegenerative diseases: Current status of drug development for Alzheimer's, Parkinson's and Huntington's diseases, and amyotrophic lateral sclerosis
by: Dunkel, P., et al.
Published: (2014)

microRNAs and Neurodegenerative Diseases
by: Qiu L., et al.
Published: (2018)

Micrornas and neurodegenerative diseases
by: Qiu L., et al.
Published: (2018)

STRUCTURES OF TDP-43 DOMAINS AND THEIR INTER-DOMAIN INTERACTIONS AS DECODED BY NMR SPECTRASCOPY
by: WEI YUANYUAN
Published: (2019)

Application of Arbuscular Mycorrhizal Fungi for Growth Enhancement of Tectona grandis Linn.f. and Aquilaria crassna Pierre ex Lec.
by: Amornrat Chaiyasen
Published: (2017)

Effect of overexpression of wild-type and mutant CU/ZN-superoxide dismutases on oxidative damage and antioxidant defences: Relevance to Down's syndrome and familial amyotrophic lateral sclerosis
by: Lee, M., et al.
Published: (2013)

STAT3 Regulates Mouse Neural Progenitor Proliferation and Differentiation by Promoting Mitochondrial Metabolism
by: Su, Y., et al.
Published: (2021)

MODULATING HOST RESPONSES TO ALTER LIVE-ATTENUATED FLAVIVIRAL VACCINE IMMUNOGENICITY
by: DARREN MOK ZHI LIANG
Published: (2024)