Results 91 to 100 of about 2,700,197 (262)

Early Dropped Head Syndrome In A Case With Amyotrophic Lateral Sclerosis

Annals of Indian Academy of Neurology, 2005
We Report a case of "Dropped head syndrome" [DHS] due to Amyotrophic lateral sclerosis [ALS]. A thirty seven year old gentleman was admitted with progressive limb and bulbar weakness of 10 months duration.
Shanbhogue K R, Rajesh S, Durairaj R, Mugundan K, Balasubramanian S, Gobinathan S, Velusamy S   +6 more
doaj  

A small-molecule inhibitor of SOD1-Derlin-1 interaction ameliorates pathology in an ALS mouse model

Nature Communications, 2018
Amyotrophic lateral sclerosis (ALS) is a neurological disease that leads to loss of voluntary muscle movement. Here, the authors screen for molecules that disrupt interaction between SOD1, a protein linked to ALS, and Derlin-1, and find an inhibitor that
Naomi Tsuburaya, Kengo Homma, Tsunehiko Higuchi, Andrii Balia, Hiroyuki Yamakoshi, Norio Shibata, Seiichi Nakamura, Hidehiko Nakagawa, Shin-ichi Ikeda, Naoki Umezawa, Nobuki Kato, Satoshi Yokoshima, Masatoshi Shibuya, Manabu Shimonishi, Hirotatsu Kojima, Takayoshi Okabe, Tetsuo Nagano, Isao Naguro, Keiko Imamura, Haruhisa Inoue, Takao Fujisawa, Hidenori Ichijo   +21 more
doaj   +1 more source

Antiviral Immune Response as a Trigger of FUS Proteinopathy in Amyotrophic Lateral Sclerosis

Cell Reports, 2019
Summary: Mutations in the FUS gene cause familial amyotrophic lateral sclerosis (ALS-FUS). In ALS-FUS, FUS-positive inclusions are detected in the cytoplasm of neurons and glia, a condition known as FUS proteinopathy.
Tatyana A. Shelkovnikova, Haiyan An, Lucy Skelt, John S. Tregoning, Ian R. Humphreys, Vladimir L. Buchman   +5 more
doaj   +1 more source

Association of NFE2L2 and KEAP1 haplotypes with amyotrophic lateral sclerosis.

, 2014
Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron syndrome influenced by oxidative stress. The transcription factor Nrf2 and its repressor Keap1 constitute an important defence system in cellular protection against oxidative stress. Here
Nilsson, Staffan, Nilsson, Ann-Charloth,, Bergstr\uf6m, Petra, Zetterberg, Henrik,, Zetterberg, Henrik, Nilsson, Staffan,, Hammarsten, Ola, von Otter, Malin,, Andersen, Peter,, Bergström, Petra,, Nilsson, Michael,, Andersen, Peter M, Nilsson, Michael, Bergström, Petra, Nilsson, Ann-Charloth, Hammarsten, Ola,, Andersen, Peter M,, von Otter, Malin   +17 more
core   +1 more source

AI‐Powered, Temperature‐Resolved Centrifugal Microfluidics for Rapid 3D Phase‐Diagram Generation of Biomolecular Condensates

Advanced Science, EarlyView.
By merging centrifugal microfluidics, precise temperature control, and AI image recognition, T‐PhaseMap transforms biomolecular condensate phase mapping into a rapid, temperature‐resolved workflow. It generates 3D composition–temperature phase diagrams within 30 min, resolves four phase states, and uncovers temperature‐dependent heparin sodium ...
Jiashuo Li, Pengjie Li, Kaiting Zhang, Yongqi Guo, Peng Chen, Bi‐Feng Liu, Yiwei Li   +6 more
wiley   +1 more source

A case of motor neuron involvement in Gaucher disease

Molecular Genetics and Metabolism Reports, 2019
Gaucher disease (GD) is a genetic disorder characterized by an accumulation of glucosylceramide in cells in the monocyte-macrophage system. We describe a case of a 33-year-old man with a previous diagnosis of type 3 GD who displayed a progressive ...
V. Pozzilli, F. Giona, M. Ceccanti, C. Cambieri, V. Frasca, E. Onesti, L. Libonati, S. Di Bari, I. Fiorini, L. Cardarelli, M. Santopietro, M. Inghilleri   +11 more
doaj   +1 more source

Matrix Rigidity Mechanoprimes Microglia for Inflammation Through Cytoskeletal‐to‐Nuclear Signaling and 3D Spatio‐Epigenomic Remodeling

Advanced Science, EarlyView.
Pathological tissue rigidity mechanoprimes microglia by enhancing actin cytoskeleton–nucleus coupling and chromatin opening at rigidity‐responsive cis‐regulatory elements (mechanoCREs). Subsequent NF‐κB/p65 signaling converges on this permissive regulatory state to amplify inflammatory gene expression and microglial activation.
Yu Xuan Meng, Jaegeon Joo, Yu Meng Li, Cheng Ji Li, Ali Taghizadeh, Seung Jae Shin, Buuvee Bayarkhangai, Tae‐Woo Ha, Sak Lee, Jaewoo Chung, Bo‐Eun Yoon, Hye Sung Kim, Inkyung Jung, Jung‐Hwan Lee, Kam W. Leong, Hae‐Won Kim   +15 more
wiley   +1 more source

Extracellular Vesicle Packaged TDP43 Derived From ECs Exacerbates Cigarette Tar‐Related Atherosclerosis Progression via Enhancing Macrophage Extracellular Traps

Advanced Science, EarlyView.
Cigarette tar triggers EC‐derived EVs carrying TDP43, which binds VDAC1 to boost MAMs, mitochondrial Ca2+ overload, and METs formation in macrophages, accelerating smoking‐related AS progression, while retinoic acid binds TDP43 to block this pathogenic cascade.
Xinxin Zhu, Yuwu Chen, Wei Meng, Biyi Xu, Junke Mou, Xiaolin Liu, Mengyang Wang, Qishuo Gu, Qianhui Sun, Man Li, Chen Zhao, Ming Zeng, Ying Lv, Boling Yi, Sheng Qu, Xiaoxuan Bai, Ji Li, Xing Luo, Sining Hu, Bo Yu, Haibo Jia   +20 more
wiley   +1 more source

Defining pre-symptomatic amyotrophic lateral sclerosis

, 2019
Successful treatment of neurodegenerative disease may hinge on early therapeutic intervention. This requires an understanding of early/pre-symptomatic disease, a need that is underscored by advances in antisense oligonucleotide, and viral-vector-based ...
Turner, Martin R, Benatar, Michael, Wuu, Joanne   +2 more
core   +1 more source

The Cytoplasmic MicroRNA‐Production Complex Regulates MicroRNA Sorting to Extracellular Vesicles During Synaptogenesis

Advanced Science, EarlyView.
The mechanisms of microRNA cargo sorting to extracellular vesicles, important mediators of cell communication, are not fully understood. This study reveals that the microRNA‐production machinery acts as a ‘hub’ for the sorting of microRNAs to extracellular vesicles, a process regulated by dynamic organelle interactions.
Yuzhou Zeng, Selina Luhmann, Aditi Saha, Marta Orlando, Haider Sami, Timo Glatter, Anna Antoniou   +6 more
wiley   +1 more source