Nucleolar stress in C9orf72 and sporadic ALS spinal motor neurons precedes TDP-43 mislocalization

Nucleolar stress in C9orf72 and sporadic ALS spinal motor neurons precedes TDP-43 mislocalization

Abstract Nucleolar stress has been implicated in the pathology and disease pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) from repeat expansions of GGGGCC in C9orf72 (C9-ALS/FTLD) but not in sporadic ALS (SALS). Previously we reported that antisense...

Full description

Bibliographic Details
Main Authors:	Olubankole Aladesuyi Arogundade, Sandra Nguyen, Ringo Leung, Danielle Wainio, Maria Rodriguez, John Ravits
Format:	Article
Language:	English
Published:	BMC 2021-02-01
Series:	Acta Neuropathologica Communications
Online Access:	https://doi.org/10.1186/s40478-021-01125-6

Similar Items

TDP-43 mislocalization drives neurofilament changes in a novel model of TDP-43 proteinopathy
by: Rachel Atkinson, et al.
Published: (2021-02-01)

Inflammation Induces TDP-43 Mislocalization and Aggregation.
by: Ana Sofia Correia, et al.
Published: (2015-01-01)

The role of TDP-43 mislocalization in amyotrophic lateral sclerosis
by: Terry R. Suk, et al.
Published: (2020-08-01)

Explore the role of AMPK activation in TDP-43 mislocalization and cell death
by: Li-Ming Lee, et al.
Published: (2014)

The Role of Mitochondrial Dysfunction and ER Stress in TDP-43 and C9ORF72 ALS
by: Ruxandra Dafinca, et al.
Published: (2021-04-01)

Autophagy and Its Impact on Neurodegenerative Diseases: New Roles for TDP-43 and C9orf72
by: Mauricio Budini, et al.
Published: (2017-05-01)

Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy
by: Jeannie Chew, et al.
Published: (2019-02-01)

Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration
by: Sarah Mizielinska, et al.
Published: (2017-04-01)

Cytoplasmic Human TDP-43 Mislocalization Induces Widespread Dendritic Spine Loss in Mouse Upper Motor Neurons
by: Marcus S. Dyer, et al.
Published: (2021-06-01)

Phosphorylated TDP-43 (pTDP-43) aggregates in the axial skeletal muscle of patients with sporadic and familial amyotrophic lateral sclerosis
by: Matthew D. Cykowski, et al.
Published: (2018-04-01)

TDP-43 is not a common cause of sporadic amyotrophic lateral sclerosis.
by: Rita J Guerreiro, et al.
Published: (2008-06-01)

Cerebrospinal Fluid TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis Patients with and without the C9ORF72 Hexanucleotide Expansion
by: Anna Junttila, et al.
Published: (2016-04-01)

Co-occurrence of TDP-43 mislocalization with reduced activity of an RNA editing enzyme, ADAR2, in aged mouse motor neurons.
by: Takuto Hideyama, et al.
Published: (2012-01-01)

TDP-43 and Limbic-Predominant Age-Related TDP-43 Encephalopathy
by: Lumi Zhang, et al.
Published: (2020-01-01)

DCTN1 Binds to TDP-43 and Regulates TDP-43 Aggregation
by: Manami Deshimaru, et al.
Published: (2021-04-01)

DNA strand breaks and TDP-43 mislocation are absent in the murine hSOD1G93A model of amyotrophic lateral sclerosis in vivo and in vitro.
by: Diane Penndorf, et al.
Published: (2017-01-01)

The D2 Dopamine Receptor Interferes With the Protective Effect of the A2A Adenosine Receptor on TDP-43 Mislocalization in Experimental Models of Motor Neuron Degeneration
by: Chia-You Lai, et al.
Published: (2018-03-01)

Chaperone Mediated Autophagy Degrades TDP-43 Protein and Is Affected by TDP-43 Aggregation
by: Fernando Ormeño, et al.
Published: (2020-02-01)

Comparative Analysis of C9orf72 and Sporadic Disease in a Large Multicenter ALS Population: The Effect of Male Sex on Survival of C9orf72 Positive Patients
by: Francesca Trojsi, et al.
Published: (2019-05-01)

HR23B pathology preferentially co-localizes with p62, pTDP-43 and poly-GA in C9ORF72-linked frontotemporal dementia and amyotrophic lateral sclerosis
by: Frederike W. Riemslagh, et al.
Published: (2019-03-01)

Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo
by: Hibiki Kawamata, et al.
Published: (2017-05-01)

TDP-43: From Alzheimer’s Disease to Limbic-Predominant Age-Related TDP-43 Encephalopathy
by: Wendi Huang, et al.
Published: (2020-02-01)

The pathological phenotypes of human TDP-43 transgenic mouse models are independent of downregulation of mouse Tdp-43.
by: Ya-Fei Xu, et al.
Published: (2013-01-01)

TDP-43 and Phosphorylated TDP-43 Levels in Paired Plasma and CSF Samples in Amyotrophic Lateral Sclerosis
by: Yuting Ren, et al.
Published: (2021-06-01)

Mechanisms and models of TDP-43 proteinopathies
by: Petrucelli Leonard
Published: (2012-02-01)

Molecular Neuropathology of TDP-43 Proteinopathies
by: Manuela Neumann
Published: (2009-01-01)

Studies of VAPB and TDP-43 in neurodegeneration
by: Müller, Sarah
Published: (2015)

Divergent phenotypes in mutant TDP-43 transgenic mice highlight potential confounds in TDP-43 transgenic modeling.
by: Simon D'Alton, et al.
Published: (2014-01-01)

Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies
by: Yohei Iguchi, et al.
Published: (2012-03-01)

Developing and Characterizing a TDP-43 Drosophila Model of Frontotemporal Dementia
by: Mathieson, Danielle Ilene, et al.
Published: (2017)

Do not curse the darkness of the spinal cord, light TDP-43
by: Kazuhide Asakawa, et al.
Published: (2021-01-01)

Spreading of TDP-43 pathology via pyramidal tract induces ALS-like phenotypes in TDP-43 transgenic mice
by: Xuebing Ding, et al.
Published: (2021-01-01)

Suppression of Conditional TDP-43 Transgene Expression Differentially Affects Early Cognitive and Social Phenotypes in TDP-43 Mice
by: Pablo R. Silva, et al.
Published: (2019-04-01)

A Novel Supplement Attenuates Oxidative Stress-Induced TDP-43-Related Pathogenesis in TDP-43-Expressed Cells
by: Eun Jin Yang
Published: (2021-01-01)

Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism
by: Erin G Conlon, et al.
Published: (2018-07-01)

Endocytosis regulates TDP-43 toxicity and turnover
by: Guangbo Liu, et al.
Published: (2017-12-01)

TDP-43 overexpression impairs presynaptic integrity
by: Lanier Heyburn, et al.
Published: (2016-01-01)

TDP-43 domain structure and aggregation studies
by: 王宜婷
Published: (2011)

Study of the Role of TDP-43 in Chromatin Organization
by: Hung-Pei Li, et al.
Published: (2013)

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