Optogenetic induction of TDP-43 aggregation impairs neuronal integrity and behavior in Caenorhabditis elegans

Abstract Background Cytoplasmic aggregation of TAR DNA binding protein 43 (TDP-43) in neurons is one of the hallmarks of TDP-43 proteinopathy. Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are closely associated with TDP-43 proteinopathy; however, it remains uncert...

Full description

Bibliographic Details
Published in:	Translational Neurodegeneration
Main Authors:	Kyung Hwan Park, Euihyeon Yu, Sooji Choi, Sangyeong Kim, Chanbin Park, J. Eugene Lee, Kyung Won Kim
Format:	Article
Language:	English
Published:	BMC 2025-04-01
Subjects:	Neurodegenerative diseases Optogenetics OptoDroplet TDP-43 proteinopathy Amyotrophic lateral sclerosis Frontotemporal lobar degeneration
Online Access:	https://doi.org/10.1186/s40035-025-00480-x

Similar Items

Heterogeneity of cortical pTDP-43 inclusion morphologies in amyotrophic lateral sclerosis
by: Rachel H. Tan, et al.
Published: (2023-11-01)

Both cytoplasmic and nuclear accumulations of the protein are neurotoxic in Drosophila models of TDP-43 proteinopathies
by: Laetitia Miguel, et al.
Published: (2011-02-01)

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

Frontotemporal Lobar degeneration with TDP-43 presenting as progressive supranuclear palsy syndrome
by: Aya Murakami, et al.
Published: (2025-07-01)

The Pathobiology of TDP-43 C-Terminal Fragments in ALS and FTLD
by: Britt A. Berning, et al.
Published: (2019-04-01)

RNA-binding deficient TDP-43 drives cognitive decline in a mouse model of TDP-43 proteinopathy
by: Julie C Necarsulmer, et al.
Published: (2023-10-01)

Methylome analysis of FTLD patients with TDP-43 pathology identifies epigenetic signatures specific to pathological subtypes
by: Cristina T. Vicente, et al.
Published: (2025-07-01)

Progranulin reduces insoluble TDP-43 levels, slows down axonal degeneration and prolongs survival in mutant TDP-43 mice
by: Sander Beel, et al.
Published: (2018-10-01)

Optogenetic TDP-43 nucleation induces persistent insoluble species and progressive motor dysfunction in vivo
by: Charlton G. Otte, et al.
Published: (2020-12-01)

A C-Terminally Truncated TDP-43 Splice Isoform Exhibits Neuronal Specific Cytoplasmic Aggregation and Contributes to TDP-43 Pathology in ALS
by: Marc Shenouda, et al.
Published: (2022-06-01)

Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors
by: Luigi Fiondella, et al.
Published: (2023-04-01)

Diffuse argyrophilic grain disease with TDP-43 proteinopathy and neuronal intermediate filament inclusion disease: FTLD with mixed tau, TDP-43 and FUS pathologies
by: Shunsuke Koga, et al.
Published: (2023-07-01)

TDP-43-M323K causes abnormal brain development and progressive cognitive and motor deficits associated with mislocalised and increased levels of TDP-43
by: Juan M. Godoy-Corchuelo, et al.
Published: (2024-04-01)

Looking into Abnormal Co-Expressions of Tau and TDP-43 in the Realm of Mixed Dementia Types: A Double-Punch Scenario
by: Hossam Youssef, et al.
Published: (2025-07-01)

Prion-like Spreading of Disease in TDP-43 Proteinopathies
by: Emma Pongrácová, et al.
Published: (2024-11-01)

TDP-43 Proteinopathy Specific Biomarker Development
by: Isabell Cordts, et al.
Published: (2023-02-01)

Multimarker synaptic protein cerebrospinal fluid panels reflect TDP-43 pathology and cognitive performance in a pathological cohort of frontotemporal lobar degeneration
by: Alba Cervantes González, et al.
Published: (2022-04-01)

Long noncoding RNAs in TDP-43 and FUS/TLS-related frontotemporal lobar degeneration (FTLD)
by: Guinevere F. Lourenco, et al.
Published: (2015-10-01)

Cyclin F can alter the turnover of TDP-43
by: Stephanie L. Rayner, et al.
Published: (2024-03-01)

Aberrant TDP‐43 phosphorylation: a key wind gap from TDP‐43 to TDP‐43 proteinopathy
by: Zi‐Qi Huang, et al.
Published: (2021-06-01)

Increase in wasteosomes (corpora amylacea) in frontotemporal lobar degeneration with specific detection of tau, TDP-43 and FUS pathology
by: Raquel Alsina, et al.
Published: (2024-06-01)

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)

CK1δ/ε kinases regulate TDP-43 phosphorylation and are therapeutic targets for ALS-related TDP-43 hyperphosphorylation
by: Vivian I. Ko, et al.
Published: (2024-06-01)

Incidence and morphology of secondary TDP-43 proteinopathies: Part 2
by: Albert Acewicz, et al.
Published: (2023-07-01)

Incidence and morphology of secondary TDP-43 proteinopathies: Part 1
by: Albert Acewicz, et al.
Published: (2022-11-01)

Neuron loss and degeneration in the progression of TDP-43 in frontotemporal lobar degeneration
by: Ahmed Yousef, et al.
Published: (2017-09-01)

Pathogenesis of Frontotemporal Lobar Degeneration: Insights From Loss of Function Theory and Early Involvement of the Caudate Nucleus
by: Gen Sobue, et al.
Published: (2018-07-01)

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)

Mitochondrion-Dependent Cell Death in TDP-43 Proteinopathies
by: Chantal B. Lucini, et al.
Published: (2021-04-01)

A panel of TDP-43-regulated splicing events verifies loss of TDP-43 function in amyotrophic lateral sclerosis brain tissue
by: Maize C. Cao, et al.
Published: (2023-09-01)

TDP-43 as a potential retinal biomarker for neurodegenerative diseases
by: Margit Glashutter, et al.
Published: (2025-02-01)

Alterations in stress granule dynamics driven by TDP-43 and FUS: A link to pathological inclusions in ALS?
by: Anais eAulas, et al.
Published: (2015-10-01)

Molecular mechanisms and consequences of TDP-43 phosphorylation in neurodegeneration
by: Elise A. Kellett, et al.
Published: (2025-05-01)

In vivo diagnosis of TDP-43 proteinopathies: in search of biomarkers of clinical use
by: Juan I. López-Carbonero, et al.
Published: (2024-06-01)

TDP-43 overexpression in the hypothalamus drives neuropathology, dysregulates metabolism and impairs behavior in mice
by: Sofia Bergh, et al.
Published: (2025-05-01)

New Antibody-Free Mass Spectrometry-Based Quantification Reveals That C9ORF72 Long Protein Isoform Is Reduced in the Frontal Cortex of Hexanucleotide-Repeat Expansion Carriers
by: Arthur Viodé, et al.
Published: (2018-08-01)

Lycorine protects motor neurons against TDP-43 proteinopathy-induced degeneration in cross-species models with amyotrophic lateral sclerosis
by: Jing Wen, et al.
Published: (2024-12-01)

Simple models to understand complex disease: 10 years of progress from Caenorhabditis elegans models of amyotrophic lateral sclerosis and frontotemporal lobar degeneration
by: Randall J. Eck, et al.
Published: (2024-01-01)

Neuronal models of TDP-43 proteinopathy display reduced axonal translation, increased oxidative stress, and defective exocytosis
by: Alessandra Pisciottani, et al.
Published: (2023-11-01)

c-Abl Regulates the Pathological Deposition of TDP-43 via Tyrosine 43 Phosphorylation
by: Saebom Lee, et al.
Published: (2022-12-01)