Detecting the Undetectable: Exploring the Diagnostic Potential of Protein Misfolding Cyclic Amplification in Human Prion Diseases [0.03%]
探索蛋白质错误折叠循环放大在人类朊病毒疾病诊断中的潜力：检测不可检测的的可能性
Federico Angelo Cazzaniga,Giuseppe Bufano,Floriana Bellandi et al.
Federico Angelo Cazzaniga et al.
Prion diseases (PrDs) are devastating and fatal conditions characterized by the accumulation of the misfolded prion protein (PrPSc) in the central nervous system (CNS). Definitive diagnosis of PrDs relies on the detection of prions in CNS t...
Advancing Prion Diagnostics: RT-QuIC Applications in Peripheral Tissues [0.03%]
进展朊病毒诊断技术：RT-QuIC在外周组织中的应用
Merve Begüm Bacınoğlu,Giuseppe Bufano,Federico Angelo Cazzaniga et al.
Merve Begüm Bacınoğlu et al.
Prion diseases (PrDs) are fatal neurodegenerative disorders characterized by the accumulation of misfolded prion protein (PrPSc) in the central nervous system (CNS). This pathological isoform of the cellular prion protein drives disease pat...
Human Genetic Evidence for New Targets in Prion Diseases: Opportunities and Challenges [0.03%]
人类遗传学证据揭示出朊病毒病的新靶点：机会与挑战
Elizabeth Hill,Simon Mead
Elizabeth Hill
Human genetics offers a powerful, causally grounded approach to therapeutic target identification in prion diseases. In this chapter, we examine strategies used to discover and evaluate gene candidates, emphasizing the complementary roles o...
Biological Fluid Biomarkers in Human Prion Diseases with a Note on Biosafety [0.03%]
人的朊毒病的生物流体生物标志物及关于生物安全性的说明注意事项
Isidro Ferrer
Isidro Ferrer
The definitive diagnosis of human prion diseases can only be obtained postmortem by combining clinical symptoms, neuropathology and PrP immunohistochemistry, Western blotting of PrP types, zygosity of codon 129, and genetic study of PRNP. P...
Reactive Microglia and Astrocytes as Therapeutic Targets in Prion Diseases [0.03%]
反应性小胶质细胞和星形胶质细胞作为朊病毒疾病的治疗靶点
Natallia Makarava,Rajesh Kushwaha,Ilia V Baskakov
Natallia Makarava
Prion diseases, also known as transmissible spongiform encephalopathies, are a group of fatal, transmissible neurodegenerative disorders affecting both humans and animals, with no available therapeutic treatments. Recent research highlights...
Christina D Orrù,Bradley R Groveman,Byron Caughey
Christina D Orrù
Prion seed amplification assays, such as real-time quaking-induced conversion (RT-QuIC), are providing ultrasensitive, specific, and increasingly practical means of detecting prions and diagnosing prion diseases in humans and other animals....
Prion and "Prion-Like" Detection: From Conventional Methods to Microfluidics or Lab-on-Chip Platforms to Monitor Seeding and Spreading of Misfolded Proteins [0.03%]
朊毒性和“朊病毒样”检测：从传统方法到微流控或芯片实验室平台以监测错误折叠蛋白的种子和传播效应
José A Del Río,Laia Lidón,Rosalina Gavín
José A Del Río
Misfolded protein neurodegeneration includes several pathologies characterized by the accumulation of a group of proteins that can modify their folding due to intrinsic or extrinsic factors, leading to the generation of aberrant forms chara...
Maria Letizia Barreca,Emiliano Biasini
Maria Letizia Barreca
Prion diseases are rare yet devastating neurodegenerative disorders that result from the misfolding of the cellular prion protein, PrPC, into its infectious and pathogenic isoform, PrPSc. These diseases are marked by progressive neuronal da...
Laura J Ellett,Matteo Senesi,Steven J Collins et al.
Laura J Ellett et al.
A typical feature of human prion diseases (PrDs) is the rapid decline to terminal illness that patients experience after symptom onset, with the most common phenotype, sporadic Creutzfeldt-Jakob disease (sCJD), frequently progressing from f...
Alberto Pettinari,Elisa Uliassi,Maria Laura Bolognesi
Alberto Pettinari
Prion diseases are fatal neurodegenerative disorders for which no effective therapies exist. Despite decades of drug discovery efforts, progress in developing disease-modifying treatments has been slow. However, recent advances have introdu...