AI Article Synopsis
Article Abstract
Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disease caused by a polyglutamine (polyQ) expansion in the androgen receptor (AR). Despite the fact that the monogenic cause of SBMA has been known for nearly 3 decades, there is no effective treatment for this disease, underscoring the complexity of the pathogenic mechanisms that lead to a loss of motor neurons and muscle in SBMA patients. In the current review, we provide an overview of the system-wide clinical features of SBMA, summarize the structure and function of the AR, discuss both gain-of-function and loss-of-function mechanisms of toxicity caused by polyQ-expanded AR, and describe the cell and animal models utilized in the study of SBMA. Additionally, we summarize previously conducted clinical trials which, despite being based on positive results from preclinical studies, proved to be largely ineffective in the treatment of SBMA; nonetheless, these studies provide important insights as researchers develop the next generation of therapies.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6985201 | PMC |
| http://dx.doi.org/10.1007/s13311-019-00790-9 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Novel functional hydrogels based on lignin‑silver nanoparticles with adhesion, antimicrobial, antioxidant and anti-freezing properties for wound dressings and pressure strain sensors.
Int J Biol Macromol
December 2024
Liaoning Province Key Laboratory of Pulp and Papermaking Engineering, Liaoning Key Lab of Lignocellulose Chemistry and BioMaterials, Liaoning Collaborative Innovation Center for Lignocellulosic Biorefinery, Dalian Polytechnic University, Dalian, Liaoning Province 116034, PR China. Electronic address:
As wound dressings and wearable electronics advance, it is critical to develop an efficacious strategy for integrating a variety of powerful functions into hydrogels. In this work, sodium lignosulfonate‑silver nanoparticles and the functional [2-(methacryloyloxy) ethyl] dimethyl-(3-sulfopropyl) ammonium hydroxide structure (SBMA) are introduced into the multifunctional lignin-based hydrogel system. The sodium lignosulfonate‑silver nanoparticles, by catalyzing multiple redox reactions, facilitate the swift curing of hydrogels at room temperature.
View Article and Find Full Text PDFZwitterionic Polymer Binder Networks with Structural Locking and Ionic Regulation Functions for High Performance Silicon Anodes.
ACS Appl Mater Interfaces
December 2024
School of Materials Science and Engineering, State Key Laboratory of Fine Chemicals, Frontiers Science Center for Smart Materials Oriented Chemical Engineering, Technology Innovation Center of High Performance Resin Materials (Liaoning Province), Dalian University of Technology, Dalian 116024, China.
The silicon anode suffers from significant volume expansion, low electrical conductivity, and poor long-term cycling performance, which collectively limit its potential to replace graphite as the anode material for lithium-ion batteries. In this article, a PAA-p(HEA-SBMA) binder was prepared by an in situ thermal cross-linking method, which combines strong mechanical properties and excellent reaction kinetics. The synergy of covalent bonding, dynamic hydrogen bonding, and ionic interactions in the binder structure provides excellent mechanical strength, which effectively dissipates stresses and "locks" the entire structure.
View Article and Find Full Text PDFFabrication of nanoparticle-reinforced composite hydrogel for improved durability, antifouling, and thrombosis-resistance in arteriovenous grafts.
Colloids Surf B Biointerfaces
November 2024
School of Nano-Tech and Nano-Bionics, University of Science and Technology of China (USTC), Hefei 230026, PR China; CAS Key Laboratory for Nano-Bio Interface, Division of Nanobiomedicine, Suzhou Institute of Nano-Tech and Nano-Bionics (SINANO), Chinese Academy of Sciences, Suzhou 215123, PR China. Electronic address:
Article Synopsis
- Arteriovenous grafts, used in hemodialysis, often face serious issues like thrombosis and infections that hinder their effectiveness.
- Researchers created a surface-modified nanohybrid hydrogel by combining clay nanoparticles coated with zwitterionic sulfobetaine methacrylate onto a sodium alginate/polyvinyl alcohol base.
- This new hydrogel demonstrated improved mechanical strength, biocompatibility, and antifouling properties, making it a promising option for use in blood-contacting implants.
Facile Preparation of Polymerizable Deep Eutectic Solvents Under Mild Conditions for Multisensory Ionic Skins.
Small
December 2024
College of Polymer Science and Engineering, State Key Laboratory of Polymer Materials Engineering, Sichuan University, Chengdu, 610065, China.
Polymerizable deep eutectic solvents (PDESs) have emerged as promising building blocks for next-generation eutectic gels, offering new opportunities for the development of advanced electronic devices. Traditional PDES fabrication typically involves heating and extended processing time. In this study, a facile method where a solid-solid mixture of lithium bis(trifluoromethane) sulfonimide (LiTFSI) and acrylamide (AAm) rapidly forms a PDES at room temperature, significantly simplifying the ionogel preparation is presented.
View Article and Find Full Text PDFHydrogel biomimetic skin inspired by human skin for resisting bacterial infection.
Biomater Adv
March 2025
Department of Basic, Zhejiang Pharmaceutical University, Ningbo 315500, PR China. Electronic address:
The flexible surface and chemical compatibility of hydrogels render them particularly appealing for research and development in antibacterial materials. However, designing tough hydrogels with multiple antibacterial mechanisms simultaneously remains a challenge. Inspired by the human skin, a hydrogel with bacterial antifouling, detection, and inactivation functions has been prepared using zwitterionic [2-(methylacrylyl) ethyl] dimethyl-(3-propyl sulfonate) ammonium hydroxide (SBMA) as the matrix and cadmium telluride quantum dots functionalised with cysteamine (CA-CdTe QDs) as the filler through micelle copolymerisation technology, achieving the integration of multiple antimicrobial mechanisms.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!