Results 1 to 10 of about 25,835 (240)

Aberrant TDP‐43 phosphorylation: a key wind gap from TDP‐43 to TDP‐43 proteinopathy [PDF]

Ibrain, 2021
TDP‐43 proteinopathy is a kind of neurodegenerative diseases related to the TAR DNA‐binding protein of 43‐kDa molecular weight (TDP‐43). The typical neurodegenerative diseases include amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration
Zi‐Qi Huang, Zhi‐Sheng Ba, Nan‐Qu Huang, Yuan‐Yuan Li, Yong Luo   +4 more
doaj   +3 more sources

TDP-43 and Limbic-Predominant Age-Related TDP-43 Encephalopathy [PDF]

Frontiers in Aging Neuroscience, 2020
Through a number of an extensive autopsy, biomarker, and genomics studies, researchers have recently defined a novel type of dementia known as limbic-predominant age-related TDP-43 encephalopathy (LATE). LATE is perhaps best characterized by the presence
Lumi Zhang, Yi Chen, Min Liu, Min Liu, Yunyun Wang, Yunyun Wang, Guoping Peng   +6 more
doaj   +3 more sources

Progressive motor weakness in transgenic mice expressing human TDP-43

Neurobiology of Disease, 2010
Familial ALS patients with TDP-43 gene mutations and sporadic ALS patients share common TDP-43 neuronal pathology. To delineate mechanisms underlying TDP-43 proteinopathies, transgenic mice expressing A315T, M337V or wild type human TDP-43 were generated.
Nancy Stallings, Dennis K Burns, Jeffrey L Elliott   +2 more
exaly   +3 more sources

Comparative effects of TDP-43-A and TDP-43-α on hippocampal subfields and amygdala nuclei. [PDF]

J Alzheimers Dis
Transactive response DNA-binding protein of 43 kDa (TDP-43) type-A is associated with frontotemporal lobar degeneration (FTLD). In primary age-related tauopathy (PART), TDP-type-α displays similar features to FTLD-TDP type-A. We compared antemortem MRI volumes of amygdala nuclei and hippocampal subfields between 16 PART-TDP-α and 12 FTLD-TDP-A autopsy ...
Youssef H, Gatto RG, Ghayal NB, Petersen RC, Jack CR, Reichard RR, Dickson DW, Whitwell JL, Josephs KA.   +8 more
europepmc   +3 more sources

Chaperone Mediated Autophagy Degrades TDP-43 Protein and Is Affected by TDP-43 Aggregation [PDF]

Frontiers in Molecular Neuroscience, 2020
TAR DNA binding protein 43 kDa (TDP-43) is a ribonuclear protein regulating many aspects of RNA metabolism. Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration (FTLD) are fatal neurodegenerative diseases with the presence of TDP-43 ...
Fernando Ormeño, Fernando Ormeño, Juan Hormazabal, José Moreno, Felipe Riquelme, Javiera Rios, Javiera Rios, Alfredo Criollo, Amelina Albornoz, Iván E. Alfaro, Iván E. Alfaro, Mauricio Budini, Mauricio Budini   +12 more
doaj   +4 more sources

The potential of TDP-43 PET ligands for a biological diagnosis of TDP-43 proteinopathies. [PDF]

Nat Commun
Irwin DJ.
europepmc   +3 more sources

DCTN1 Binds to TDP-43 and Regulates TDP-43 Aggregation [PDF]

International Journal of Molecular Sciences, 2021
A common pathological hallmark of several neurodegenerative diseases, including amyotrophic lateral sclerosis, is cytoplasmic mislocalization and aggregation of nuclear RNA-binding protein TDP-43. Perry disease, which displays inherited atypical parkinsonism, is a type of TDP-43 proteinopathy. The causative gene DCTN1 encodes the largest subunit of the
Manami Deshimaru, Mariko Kinoshita-Kawada, Kaori Kubota, Takuya Watanabe, Yasuyoshi Tanaka, Saito Hirano, Fumiyoshi Ishidate, Masaki Hiramoto, Mitsuru Ishikawa, Yoshinari Uehara, Hideyuki Okano, Shinichi Hirose, Shinsuke Fujioka, Katsunori Iwasaki, Junichi Yuasa-Kawada, Takayasu Mishima, Yoshio Tsuboi   +16 more
openaire   +2 more sources

Disease‐linked TDP‐43 hyperphosphorylation suppresses TDP‐43 condensation and aggregation [PDF]

The EMBO Journal, 2021
Abstract Post-translational modifications (PTMs) have emerged as key modulators of protein phase separation and have been linked to protein aggregation in neurodegenerative disorders. The major aggregating protein in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), the RNA-binding protein TDP-43, is ...
Lara Gruijs da Silva, Francesca Simonetti, Saskia Hutten, Henrick Riemenschneider, Erin L. Sternburg, Lisa M. Pietrek, Jakob Gebel, Volker Dötsch, Dieter Edbauer, Gerhard Hummer, Lukas S. Stelzl, Dorothee Dormann   +11 more
openaire   +8 more sources

TDP-43 Prions [PDF]

Cold Spring Harbor Perspectives in Medicine, 2017
The most common neurodegenerative diseases, such as Alzheimer's, Parkinson's, and amyotrophic lateral sclerosis, are all protein-misfolding diseases and are characterized by the presence of disease-specific protein aggregates in affected neuronal cells. Recent studies have shown that, like tau and α-synuclein, TAR-DNA binding protein of 43 kDa (TDP-43)
Takashi, Nonaka, Masato, Hasegawa
openaire   +2 more sources

Integration of FUNDC1-associated mitochondrial protein import and mitochondrial quality control contributes to TDP-43 degradation

Cell Death and Disease, 2023
Though TDP-43 protein can be translocated into mitochondria and causes mitochondrial damage in TDP-43 proteinopathy, little is known about how TDP-43 is imported into mitochondria.
Jinfa Ma, Lei Liu, Lu Song, Jianghong Liu, Lingyao Yang, Quan Chen, Jane Y. Wu, Li Zhu   +7 more
doaj   +1 more source