Results 1 to 10 of about 41,959 (160)

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   +10 more sources

C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress [PDF]

Molecular Neurodegeneration, 2011
Background TDP-43 proteinopathies are characterized by loss of nuclear TDP-43 expression and formation of C-terminal TDP-43 fragmentation and accumulation in the cytoplasm. Recent studies have shown that TDP-43 can accumulate in RNA stress granules (SGs)
Masters Colin L, Li Qiao-Xin, Caragounis Aphrodite, Liddell Jeffrey R, Price Katherine A, Ng Dominic CH, Vella Laura J, Parker Sarah J, Meyerowitz Jodi, Nonaka Takashi, Hasegawa Masato, Bogoyevitch Marie A, Kanninen Katja M, Crouch Peter J, White Anthony R   +14 more
doaj   +3 more sources

Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice [PDF]

Molecular Neurodegeneration, 2011
Background Abnormal distribution, modification and aggregation of transactivation response DNA-binding protein 43 (TDP-43) are the hallmarks of multiple neurodegenerative diseases, especially frontotemporal lobar degeneration with ubiquitin-positive ...
Dickson Dennis W, Stetler Caroline, Cao Xiangkun, Lin Wen-Lang, Zhang Yong-Jie, Xu Ya-Fei, Lewis Jada, Petrucelli Leonard   +7 more
doaj   +4 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

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

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 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

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
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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
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Sustained therapeutic benefits by transient reduction of TDP-43 using ENA-modified antisense oligonucleotides in ALS/FTD mice

Molecular Therapy: Nucleic Acids, 2023
The abnormal aggregation of TDP-43 into cytoplasmic inclusions in affected neurons is a pathological hallmark of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).
Toshihide Takeuchi, Kazuhiro Maeta, Xin Ding, Yukako Oe, Akiko Takeda, Mana Inoue, Seiichi Nagano, Tsuyoshi Fujihara, Seiji Matsuda, Shinsuke Ishigaki, Kentaro Sahashi, Eiko N. Minakawa, Hideki Mochizuki, Masahiro Neya, Gen Sobue, Yoshitaka Nagai   +15 more
doaj   +1 more source