Results 71 to 80 of about 72,088 (314)

Colocalization of 14-3-3 proteins with SOD1 in Lewy body-like hyaline inclusions in familial amyotrophic lateral sclerosis cases and the animal model. [PDF]

PLoS ONE, 2011
Cu/Zn superoxide dismutase (SOD1) is a major component of Lewy body-like hyaline inclusion (LBHI) found in the postmortem tissue of SOD1-linked familial amyotrophic lateral sclerosis (FALS) patients. In our recent studies, 14-3-3 proteins have been found
Yoko Okamoto, Yoshitomo Shirakashi, Masafumi Ihara, Makoto Urushitani, Miki Oono, Yasuhiro Kawamoto, Hirofumi Yamashita, Shun Shimohama, Shinsuke Kato, Asao Hirano, Hidekazu Tomimoto, Hidefumi Ito, Ryosuke Takahashi   +12 more
doaj   +1 more source

Protein trafficking in the mitochondrial intermembrane space: mechanisms and links to human disease [PDF]

, 2017
Mitochondria fulfill a diverse range of functions in cells including oxygen metabolism, homeostasis of inorganic ions and execution of apoptosis. Biogenesis of mitochondria relies on protein import pathways that are ensured by dedicated multiprotein ...
MacPherson, Lisa, Tokatlidis, Konstantinos   +1 more
core   +1 more source

Amyotrophic Lateral Sclerosis Prevalence Projection in 2040: A Less Rare Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To project ALS prevalence across multiple countries through 2040, accounting for both population aging and increased survival. Methods Data from the Piemonte and Valle d'Aosta ALS register (PARALS) was used to estimate the trends in incidence and prevalence from 2005 to 2019. Survival trends over this period were also assessed.
Rosario Vasta, Stefano Callegaro, Antonio Canosa, Umberto Manera, Maurizio Grassano, Francesca Palumbo, Sara Cabras, Enrico Matteoni, Francesca Di Pede, Filippo De Mattei, Salvatore Tafaro, Neil M. Thakur, Ryan Grosenick, Fabiola De Marchi, Letizia Mazzini, Cristina Moglia, Andrea Calvo, Kuldip D. Dave, Adriano Chiò   +18 more
wiley   +1 more source

Susceptibility to ulcerative colitis and genetic polymorphisms of A251G SOD1 and C-262T CAT [PDF]

Journal of Medical Biochemistry, 2016
Background: Reactive oxygen species can attack and damage almost every molecule found in living cells, including proteins, carbohydrates, lipids, and DNA. For this reason, their production is normally tightly controlled. Among the most important defenses
El-Kheshen Gadier, Moeini Maryam, Saadat Mostafa   +2 more
doaj  

Expression of Human SOD1 and Mutant SOD1 (G93A) in E. coli and Identification of SOD1 as a Substrate of HtrA2 Serine Protease [PDF]

Journal of Life Science, 2006
Superoxide dismutase (SOD) is physiologically important in regulating cellular homeostasis and apoptotic cell death, and its mutations are the cause of familial amyotrophic lateral sclerosis (FALS). Mitochondrial serine protease HtrA2 has a pro-apoptotic function and has known to be associated with neurodegenerative disorders.
Sangsoo Kim, Hyangshuk Rhim, Goo-Young Kim, Hyo Jin Park   +3 more
openaire   +2 more sources

FeDSNP‐Pa Nanoassemblies: A Triple‐Action Therapeutic Strategy Targeting Oxidative Stress, Inflammation, and Pyroptosis for Retinal Ganglion Cell Protection in Glaucoma

Advanced Functional Materials, EarlyView.
FeDSNP‐Pa, a metallized nanoparticle loaded with sodium pyruvate (Pa), exerts triple therapeutic effects by scavenging reactive oxygen species (ROS), suppressing inflammatory responses, and inhibiting pyroptosis signaling pathways. This multifunctional neuroprotective strategy protecting retinal ganglion cells (RGCs) from elevated intraocular pressure ...
Yukun Wu, Xue Li, Tong Wu, Dan Ji, Yugang Bai, Xuezhi Zhou   +5 more
wiley   +1 more source

Redox susceptibility of SOD1 mutants is associated with the differential response to CCS over-expression in vivo

Neurobiology of Disease, 2009
Over-expression of CCS in G93A SOD1 mice accelerates neurological disease and enhances mitochondrial pathology. We studied the effect of CCS over-expression in transgenic mice expressing G37R, G86R or L126Z SOD1 mutations in order to understand factors ...
Marjatta Son, Qiao Fu, Krishna Puttaparthi, Christina M. Matthews, Jeffrey L. Elliott   +4 more
doaj   +1 more source

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. [PDF]

, 2010
Many mutations confer one or more toxic function(s) on copper/zinc superoxide dismutase 1 (SOD1) that impair motor neuron viability and cause familial amyotrophic lateral sclerosis (FALS).
A Durazo, A Gruzman, A Kerman, A Tiwari, AG Estévez, AK Svensson, AL Ström, Benjamin A Fontaine, C Vande Velde, Daryl A Bosco, DE Bredesen, Diane McKenna-Yasek, E Kabashi, F Gros-Louis, Francois Gros-Louis, G Morfini, G Morfini, GA Morfini, GA Morfini, Gerardo Morfini, HN Liu, Holly Goolsby, Jean-Pierre Julien, Jeffrey N Agar, JF Collard, JS Beckman, JS Valentine, KJ De Vos, KS Molnar, L Banci, L Munoz, L Wang, LI Bruijn, LJ Hayward, LR Fischer, M Chattopadhyay, M Prudencio, M Urushitani, M Urushitani, M Watanabe, MA Fabian, Matthew P Frosch, MJ Lindberg, N Fujiwara, N Murat Karabacak, N Shibata, N Shibata, Nathan Lemay, P Pasinelli, Piera Pasinelli, PN Valdmanis, Q Wang, R Rakhit, R Rakhit, R Rakhit, R Tandan, Robert H Brown, RW Strange, SA Ezzi, Scott T Brady, ST Brady, ST Brady, TL Williamson, Y Furukawa, Yuyu Song   +64 more
core   +2 more sources

Quantitative Profiling of Nanoscopic Protein Aggregates Reveals Specific Fingerprint of TDP‐43‐Positive Assemblies in Motor Neuron Disease

Advanced Science, EarlyView.
Single‐molecule fluorescence microscopy is combined with proteomic profiling to fingerprint the morphology and composition of TDP‐43 protein aggregates found in post‐mortem motor neuron disease brain tissues. Aggregate fingerprints can distinguish disease from control donors and detect unexpected TDP‐43 pathology in SOD1‐MND.
Dezerae Cox, Melanie Burke, Sara Milani, Matthew A White, Fergal M Waldron, Dorothea Böken, Evgeniia Lobanova, Jemeen Sreedharan, Jenna M Gregory, David Klenerman   +9 more
wiley   +1 more source

Inflammation mobilizes copper metabolism to promote colon tumorigenesis via an IL-17-STEAP4-XIAP axis. [PDF]

, 2020
Copper levels are known to be elevated in inflamed and malignant tissues. But the mechanism underlying this selective enrichment has been elusive. In this study, we report a axis by which inflammatory cytokines, such as IL-17, drive cellular copper ...
Bao, Shideng, Bulek, Katarzyna, Cai, Gang, Chang, Christopher J, Chen, Xing, Fox, Paul L, Huang, Emina, Jackson, Mark W, Jia, Shang, Kalady, Matthew F, Li, Xiaoxia, Liao, Yun, Pizarro, Theresa T, Sen, Ganes C, Stark, George R, Tang, Fangqiang, Zhao, Junjie   +16 more
core