Results 71 to 80 of about 14,543 (225)

Gray matter changes in asymptomatic C9orf72 and GRN mutation carriers

NeuroImage: Clinical, 2018
Frontotemporal dementia (FTD) is a neurodegenerative disease with a strong genetic basis. Understanding the structural brain changes during pre-symptomatic stages may allow for earlier diagnosis of patients suffering from FTD; therefore, we investigated ...
Karteek Popuri, Emma Dowds, Mirza Faisal Beg, Rakesh Balachandar, Mahadev Bhalla, Claudia Jacova, Adrienne Buller, Penny Slack, Pheth Sengdy, Rosa Rademakers, Dana Wittenberg, Howard H. Feldman, Ian R. Mackenzie, Ging-Yuek R. Hsiung   +13 more
doaj   +1 more source

Loss of C9orf72 increases TFEB in the nucleus. [PDF]

, 2016
A) Western analysis of TFEB levels after knockdown of C9orf72 in HEK293T cells. HEK293T were transfected with GFP-tagged TFEB and C9orf72 shRNA or control shRNA and then the lysates were analyzed using an antibody to GFP.
Justin Chu (732152), Yon Ju Ji (3359057), Janet Ugolino (3359054), Jiou Wang (267956), Karen Conchina (3359060), Akhilesh Pandey (14293), Raja Sekhar Nirujogi (841149), Anne Hamacher-Brady (177723), Nathan R. Brady (51959)   +8 more
core   +1 more source

Negotiating in a Foreign Land: Understanding the Curious Interactions Between Intracellular Mitochondria and Internalized Nanoparticles

Advanced Materials Interfaces, EarlyView.
Therapeutic nano‐drug delivery systems interact with cellular mitochondria in a multitude of ways. While the complexity of such interactions disrupts the mitochondrial electron transport chain and increases reactive oxygen species production, thereby contributing to nanoparticle toxicity, they also present unique theranostic opportunities in diseases ...
Sourav Bhattacharjee
wiley   +1 more source

Mesenchymal stem cell–derived extracellular vesicle treatment of induced pluripotent stem cell–derived motor neurons with different amyotrophic lateral sclerosis genetic backgrounds

Neural Regeneration Research
Motor neurons derived from induced human pluripotent stem cells offer a powerful model to study motor neuron diseases, such as amyotrophic lateral sclerosis. While widely used, our knowledge of the proteomic changes in these models is rather rudimentary.
Suzy Varderidou-Minasian, Channa E. Jakobs, Svetlana Pasteuning-Vuhman, Lars Gal, Annabel Timmers, Maarten Altelaar, Magdalena J. Lorenowicz, R. Jeroen Pasterkamp   +7 more
doaj   +1 more source

C9orf72/ALFA-1 controls TFEB/HLH-30-dependent metabolism through dynamic regulation of Rag GTPases.

PLoS Genetics, 2020
Nutrient utilization and energy metabolism are critical for the maintenance of cellular homeostasis. A mutation in the C9orf72 gene has been linked to the most common forms of neurodegenerative diseases that include amyotrophic lateral sclerosis (ALS ...
Yon Ju Ji, Janet Ugolino, Tao Zhang, Jiayin Lu, Dohoon Kim, Jiou Wang   +5 more
doaj   +1 more source

m6A‐Mediated Glycolysis by IL‐37 Drives T Cell Metabolic Reprogramming to Regulate Colitis

Advanced Science, EarlyView.
This study identifies an IL‐37/SIGIRR‐METTL14 regulatory axis that suppresses global m6A modification in CD4+ T cells. IL‐37 signaling, mediated through SIGIRR, inhibits IRAK4 and JNK phosphorylation, leading to downregulation of the methyltransferase METTL14.
Xiaoyan Wang, Jiadong Yu, Guolin Li, Ya Li, Fanlian Zeng, Jing Hu, Yifan Zhou, Chengcheng Yue, Qixiang Zhao, Chen Zhang, Hong Zhou, Fulei Zhao, Yawen Hu, Pei Zhou, Linna Gu, Yuting Feng, Mingxiang He, Shishi Huang, Xiaochi Sun, Xinmeng Wang, Jie Tang, Xinyu Zeng, Wenling Wu, Nongyu Huang, Xinai Cui, Kaijun Cui, Jiong Li   +26 more
wiley   +1 more source

GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function

eLife, 2015
Microsatellite expansions are the leading cause of numerous neurodegenerative disorders. Here we demonstrate that GGGGCC and CAG microsatellite repeat RNAs associated with C9orf72 in amyotrophic lateral sclerosis/frontotemporal dementia and with ...
Alondra Schweizer Burguete, Sandra Almeida, Fen-Biao Gao, Robert Kalb, Michael R Akins, Nancy M Bonini   +5 more
doaj   +1 more source

C9ORF72 repeat expansion in Australian and Spanish frontotemporal dementia patients. [PDF]

PLoS ONE, 2013
A hexanucleotide repeat expansion in C9ORF72 has been established as a common cause of frontotemporal dementia (FTD). However, the minimum repeat number necessary for disease pathogenesis is not known.
Carol Dobson-Stone, Marianne Hallupp, Clement T Loy, Elizabeth M Thompson, Eric Haan, Carolyn M Sue, Peter K Panegyres, Cristina Razquin, Manuel Seijo-Martínez, Ramon Rene, Jordi Gascon, Jaume Campdelacreu, Birgit Schmoll, Alexander E Volk, William S Brooks, Peter R Schofield, Pau Pastor, John B J Kwok   +17 more
doaj   +1 more source

Molecular and Cellular Hallmarks of Age‐Related Vestibular Hair Cell Degeneration

Advanced Science, EarlyView.
This study utilizes single‐cell RNA‐seq transcriptomes, advanced imaging, and electrophysiology to examine universal and cell‐type‐specific aging signatures of vestibular hair cells. The study shows that impaired hair bundle function is a key driver of age‐related vestibular dysfunction.
Samadhi Kulasooriya, Huizhan Liu, Sarath Vijayakumar, Celia Bloom, Zhenhang Xu, Shu Tu, Benjamin J. Borgmeier, Mi Zhou, Litao Tao, Bechara Kachar, David Z. He   +10 more
wiley   +1 more source

C9orf72 regulates TFEB through Rag GTPases. [PDF]

, 2020
(A) Co-immunoprecipitation analysis of C9orf72-Flag and TFEB-GFP. HEK293 cells were transfected with the indicated plasmids, cell lysates were prepared at 48 hr post-transfection, and IgG, HA, or FLAG-tagged immunoprecipitates were analyzed by ...
Yon Ju Ji (3359057), Tao Zhang (43681), Janet Ugolino (3359054), Jiou Wang (267956), Jiayin Lu (716730), Dohoon Kim (251220)   +5 more
core   +1 more source