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The role of TDP-43 mislocalization in amyotrophic lateral sclerosis.
Suk TR, Rousseaux MWC. Suk TR, et al. Mol Neurodegener. 2020 Aug 15;15(1):45. doi: 10.1186/s13024-020-00397-1. Mol Neurodegener. 2020. PMID: 32799899 Free PMC article. Review.
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP-43) has remained a central focus to understand the disease. T …
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue of patients with Amyotrophic Lateral …
Amyotrophic lateral sclerosis caused by TARDBP mutations: from genetics to TDP-43 proteinopathy.
Balendra R, Sreedharan J, Hallegger M, Luisier R, Lashuel HA, Gregory JM, Patani R. Balendra R, et al. Lancet Neurol. 2025 May;24(5):456-470. doi: 10.1016/S1474-4422(25)00109-7. Lancet Neurol. 2025. PMID: 40252666 Free PMC article. Review.
Mutations in the TARDBP gene, which encodes the TDP-43 protein, account for only 3-5% of familial cases of amyotrophic lateral sclerosis and less than 1% of cases that are apparently idiopathic. However, the discovery of neuronal inclusions of …
Mutations in the TARDBP gene, which encodes the TDP-43 protein, account for only 3-5% of familial cases of amyotrophic …
Lost in local translation: TDP-43 and FUS in axonal/neuromuscular junction maintenance and dysregulation in amyotrophic lateral sclerosis.
Piol D, Robberechts T, Da Cruz S. Piol D, et al. Neuron. 2023 May 3;111(9):1355-1380. doi: 10.1016/j.neuron.2023.02.028. Epub 2023 Mar 23. Neuron. 2023. PMID: 36963381 Free article. Review.
Key early features of amyotrophic lateral sclerosis (ALS) are denervation of neuromuscular junctions and axonal degeneration. ...We focus on two RNA binding proteins, transactive response DNA binding protein-43 (TDP-43) and fused in sarco …
Key early features of amyotrophic lateral sclerosis (ALS) are denervation of neuromuscular junctions and axonal degener …
Pathogenic TDP-43 in amyotrophic lateral sclerosis.
Chong ZZ, Souayah N. Chong ZZ, et al. Drug Discov Today. 2025 May;30(5):104351. doi: 10.1016/j.drudis.2025.104351. Epub 2025 Apr 4. Drug Discov Today. 2025. PMID: 40188980 Free article. Review.
The aberrant expression of the transactive response DNA-binding protein of 43 kDa (TDP-43) has been closely associated with amyotrophic lateral sclerosis (ALS). Cytoplasmic inclusions containing TDP-43 can be found in the brain and …
The aberrant expression of the transactive response DNA-binding protein of 43 kDa (TDP-43) has been closely associated with …
TDP-43: A Key Therapeutic Target beyond Amyotrophic Lateral Sclerosis.
Palomo V, Tosat-Bitrian C, Nozal V, Nagaraj S, Martin-Requero A, Martinez A. Palomo V, et al. ACS Chem Neurosci. 2019 Mar 20;10(3):1183-1196. doi: 10.1021/acschemneuro.9b00026. Epub 2019 Mar 4. ACS Chem Neurosci. 2019. PMID: 30785719 Free article. Review.
Accumulation of TDP-43 in the cytoplasm of diseased neurons is the pathological hallmark of frontotemporal dementia-TDP (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), two diseases that lack efficacious medicine to prevent or to stop disease …
Accumulation of TDP-43 in the cytoplasm of diseased neurons is the pathological hallmark of frontotemporal dementia-TDP (FTLD- …
TDP-43-stratified single-cell proteomics of postmortem human spinal motor neurons reveals protein dynamics in amyotrophic lateral sclerosis.
Guise AJ, Misal SA, Carson R, Chu JH, Boekweg H, Van Der Watt D, Welsh NC, Truong T, Liang Y, Xu S, Benedetto G, Gagnon J, Payne SH, Plowey ED, Kelly RT. Guise AJ, et al. Cell Rep. 2024 Jan 23;43(1):113636. doi: 10.1016/j.celrep.2023.113636. Epub 2024 Jan 5. Cell Rep. 2024. PMID: 38183652 Free PMC article.
A limitation of conventional bulk-tissue proteome studies in amyotrophic lateral sclerosis (ALS) is the confounding of motor neuron (MN) signals by admixed non-MN proteins. ...In a follow-up analysis, we examine the impact of stratification of MNs based on cy …
A limitation of conventional bulk-tissue proteome studies in amyotrophic lateral sclerosis (ALS) is the confounding of …
TDP-43 and Inflammation: Implications for Amyotrophic Lateral Sclerosis and Frontotemporal Dementia.
Bright F, Chan G, van Hummel A, Ittner LM, Ke YD. Bright F, et al. Int J Mol Sci. 2021 Jul 21;22(15):7781. doi: 10.3390/ijms22157781. Int J Mol Sci. 2021. PMID: 34360544 Free PMC article. Review.
The abnormal mislocalisation and ubiquitinated protein aggregation of the TAR DNA binding protein 43 (TDP-43) within the cytoplasm of neurons and glia in the central nervous system (CNS) is a pathological hallmark of early-onset neurodegenerative disorders …
The abnormal mislocalisation and ubiquitinated protein aggregation of the TAR DNA binding protein 43 (TDP-43) within th …
TDP-43 proteinopathy in frontotemporal lobar degeneration and amyotrophic lateral sclerosis: From pathomechanisms to therapeutic strategies.
Ho PC, Hsieh TC, Tsai KJ. Ho PC, et al. Ageing Res Rev. 2024 Sep;100:102441. doi: 10.1016/j.arr.2024.102441. Epub 2024 Jul 27. Ageing Res Rev. 2024. PMID: 39069095 Free article. Review.
The transactivation response (TAR) DNA-binding protein 43 (TDP-43) plays a critical role as an RNA/DNA-binding protein in RNA metabolism and synaptic function. Accumulation of TDP-43 aggregates in the central nervous system is a hallmark of fron …
The transactivation response (TAR) DNA-binding protein 43 (TDP-43) plays a critical role as an RNA/DNA-binding protein …
TDP-43 Pathology in Alzheimer's Disease.
Meneses A, Koga S, O'Leary J, Dickson DW, Bu G, Zhao N. Meneses A, et al. Mol Neurodegener. 2021 Dec 20;16(1):84. doi: 10.1186/s13024-021-00503-x. Mol Neurodegener. 2021. PMID: 34930382 Free PMC article. Review.
Cytoplasmic inclusion bodies containing phosphorylated and truncated forms of TDP-43 are hallmarks of amyotrophic lateral sclerosis (ALS) and a subset of frontotemporal lobar degeneration (FTLD). ...In some cases, TDP-43 deposits a …
Cytoplasmic inclusion bodies containing phosphorylated and truncated forms of TDP-43 are hallmarks of amyotrophic la …
Drug Screening and Validation Targeting TDP-43 Proteinopathy for Amyotrophic Lateral Sclerosis.
Xin J, Huang S, Wen J, Li Y, Li A, Satyanarayanan SK, Yao X, Su H. Xin J, et al. Aging Dis. 2024 Feb 1;16(2):693-713. doi: 10.14336/AD.2024.0440. Aging Dis. 2024. PMID: 38739934 Free PMC article. Review.
Amyotrophic lateral sclerosis (ALS) stands as a rare, yet severely debilitating disorder marked by the deterioration of motor neurons (MNs) within the brain and spinal cord, which is accompanied by degenerated corticobulbar/corticospinal tracts and denervatio …
Amyotrophic lateral sclerosis (ALS) stands as a rare, yet severely debilitating disorder marked by the deterioration of …
3,389 results