Results 1 to 10 of about 45,245 (190)

The tau tubulin kinases TTBK1/2 promote accumulation of pathological TDP-43. [PDF]

PLoS Genetics, 2014
Pathological aggregates of phosphorylated TDP-43 characterize amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD-TDP), two devastating groups of neurodegenerative disease.
Nicole F Liachko, Pamela J McMillan, Timothy J Strovas, Elaine Loomis, Lynne Greenup, Jill R Murrell, Bernardino Ghetti, Murray A Raskind, Thomas J Montine, Thomas D Bird, James B Leverenz, Brian C Kraemer   +11 more
doaj   +7 more sources

scFv intrabody targeting wildtype TDP-43 presents protective effects in a cellular model of TDP-43 proteinopathy. [PDF]

PLoS ONE
TDP-43 proteinopathies are neurological disorders marked by the abnormal accumulation of TDP-43 in the cytoplasm. This mislocalization disrupts the normal function of the protein.
Yara Al Ojaimi, Rudolf Hergesheimer, Anna A Chami, Hugo Alarcan, Johanna Augros, Audrey Dangoumau, Shanez Haouari, Jérôme Bourgeais, Antoine Lefevre, Samira Osman, Patrick Emond, Patrick Vourc'h, Christian R Andres, Philippe Corcia, Olivier Herault, Pierre Martineau, Débora Lanznaster, Hélène Blasco   +17 more
doaj   +2 more sources

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

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   +1 more source

TDP-43 Oligomerization and Phase Separation Properties Are Necessary for Autoregulation

Frontiers in Neuroscience, 2022
Loss of TDP-43 protein homeostasis and dysfunction, in particular TDP-43 aggregation, are tied to amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).
Lydia C. Koehler, Zachary R. Grese, Alliny C. S. Bastos, Lohany D. Mamede, Tomasz Heyduk, Yuna M. Ayala   +5 more
doaj   +1 more source

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

RNA-binding deficient TDP-43 drives cognitive decline in a mouse model of TDP-43 proteinopathy

eLife, 2023
TDP-43 proteinopathies including frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) are neurodegenerative disorders characterized by aggregation and mislocalization of the nucleic acid-binding protein TDP-43 and subsequent ...
Julie C Necarsulmer, Jeremy M Simon, Baggio A Evangelista, Youjun Chen, Xu Tian, Sara Nafees, Ariana B Marquez, Huijun Jiang, Ping Wang, Deepa Ajit, Viktoriya D Nikolova, Kathryn M Harper, J Ashley Ezzell, Feng-Chang Lin, Adriana S Beltran, Sheryl S Moy, Todd J Cohen   +16 more
doaj   +1 more source

Evidence of cerebellar TDP-43 loss of function in FTLD-TDP

Acta Neuropathologica Communications, 2022
Frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP) is a neurodegenerative disease primarily affecting the frontal and/or temporal cortices.
Sarah Pickles, Tania F. Gendron, Yuka Koike, Mei Yue, Yuping Song, Jennifer M. Kachergus, J. Shi, Michael DeTure, E. Aubrey Thompson, Björn Oskarsson, Neill R. Graff-Radford, Bradley F. Boeve, Ronald C. Petersen, Zbigniew K. Wszolek, Keith A. Josephs, Dennis W. Dickson, Leonard Petrucelli, Casey N. Cook, Mercedes Prudencio   +18 more
doaj   +1 more source

TDP-43 in neurodegeneration and cancer: Decoding the mechanism of mRNA localization and translation [PDF]

Biochem Biophys Rep
panjiao pang, Mingyang Yu, Huixin Ma, Longkai Wei, Jiali Huang, Lingxin Zhao, Ying-hua Zhang   +6 more
europepmc   +2 more sources

TDP-43 and Limbic-Predominant Age-Related TDP-43 Encephalopathy

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   +1 more source

Degradation of neurodegenerative disease-associated TDP-43 aggregates and oligomers via a proteolysis-targeting chimera

Journal of Biomedical Science, 2023
Background Amyotrophic lateral sclerosis (ALS) associated with TAR DNA-binding protein 43 (TDP-43) aggregation has been considered as a lethal and progressive motor neuron disease.
Yu-Ling Tseng, Po-Chao Lu, Chi-Chang Lee, Ruei-Yu He, Yung-An Huang, Yin-Chen Tseng, Ting-Jen Rachel Cheng, Joseph Jen-Tse Huang, Jim-Min Fang   +8 more
doaj   +1 more source