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1. Prion Protein Conversion and Lipids

   The conversion of α-helical rich normal prion protein to a β-sheeted pathogenic isoform is central to prion disease. Decades of studies provided...

   Jiyan Ma, **angyi Zhang in Prions and Diseases

   Chapter 2023
   

2. Prion Strain Interference

   Prions are transmissible agents comprised of a misfolded protein PrPSc that is post-translationally derived from the normal isoform PrPC. Prion...

   Ronald A. Shikiya, Jason C. Bartz in Prions and Diseases

   Chapter 2023
   

3. Differential expression of cellular prion protein (PrP^C) in mouse hepatitis virus induced neuroinflammation

   The cellular prion protein (PrP ^C ) is an extracellular cell membrane protein. Due to its diversified roles, a definite role of PrP ^C has been difficult...

   Satavisha Ghosh, Rishika Jana, ... Jayasri Das Sarma in Journal of NeuroVirology

   Article 26 June 2024
   

4. Disease-Associated Q159X Mutant Prion Protein Is Sufficient to Cause Fatal Degenerative Disease in Mice

   PRNP Q160X is one of the five dominantly inheritable nonsense mutations causing familial prion diseases. Till now, it remains unclear how this type...

   Yan Zhang, Runchuan Yan, ... Jiyan Ma in Molecular Neurobiology

   Article 14 May 2024
   

5. Protective Role of Cellular Prion Protein in Tissues Ischemic/Reperfusion Injury

   Normal cellular prion protein (PrPC) is well documented to be the precursor of the infectious pathogenic prion protein that plays a critical role in...

   Zerui Wang, Wen-Quan Zou in Prions and Diseases

   Chapter 2023
   

6. PrP Prion Structures

   The biophysical properties of authentic infectious prion protein (PrP)-based mammalian prions have long impeded determination of their detailed...

   Byron Caughey, Efrosini Artikis, Allison Kraus in Prions and Diseases

   Chapter 2023
   

7. Species Barriers in Prion Disease

   Species barriers in prion diseases are defined by the difficulty that prions from one species have in triggering prion infection in a new species....

   Suzette A. Priola in Prions and Diseases

   Chapter 2023
   

8. Insoluble Cellular Prion Protein and Other Neurodegeneration-Related Protein Aggregates in the Brain of Asymptomatic Individuals

   The pathological detergent-insoluble prion protein (PrPSc) is derived from its normal detergent-soluble cellular form (PrPC) through a structural...

   Wen-Quan Zou in Prions and Diseases

   Chapter 2023
   

9. Human Prion Disease Surveillance

   Human prion diseases are characterized by rapid fatal neurodegeneration caused by pathologic prion proteins that are transmissible under specific...

   Brian S. Appleby, Lawrence B. Schonberger, Ermias D. Belay in Prions and Diseases

   Chapter 2023
   

10. Prion Conversion and Deformed Templating

    The transmissible agent of prion disease consists of a prion protein in its abnormal, β-sheet-rich state (PrPSc), which replicates itself according...

    Ilia V. Baskakov in Prions and Diseases

    Chapter 2023
    

11. Cofactor Involvement in Prion Propagation

    Pure amyloid proteins are responsible for the transmissible properties of yeast prions (Tanaka et al., Nature 428(6980):323–328, 2004; Cell...

    Surachai Supattapone, Michael B. Miller in Prions and Diseases

    Chapter 2023
    

12. Genetics of Prion Disease

    The Prion Diseases (PrDs) are rare transmissible neurodegenerative diseases that result from the accumulation of a misfolded isoform (PrPSc) of the...

    James A. Mastrianni in Prions and Diseases

    Chapter 2023
    

13. Accumulation of Prion Triggers the Enhanced Glycolysis via Activation of AMKP Pathway in Prion-Infected Rodent and Cell Models

    Mitochondrial dysfunction is one of the hallmarks in the pathophysiology of prion disease and other neurodegenerative diseases. Various metabolic...

    Qin Fan, Kang **ao, ... **ao-** Dong in Molecular Neurobiology

    Article 20 September 2023
    

14. Comparing Prion Proteins Across Species: Is Zebrafish a Useful Model?

    Despite the considerable body of research dedicated to the field of neurodegeneration, the gap in knowledge on the prion protein and its intricate...

    Anna Burato, Giuseppe Legname in Molecular Neurobiology

    Article Open access 25 June 2024
    

15. Human Sporadic Prion Diseases

    Sporadic or idiopathic prion diseases account for over 90% of all human prion diseases, and sporadic Creutzfeldt–Jakob disease (sCJD) is by far the...

    Pierluigi Gambetti, Ignazio Cali in Prions and Diseases

    Chapter 2023
    

16. Drosophila Models of Prion Diseases

    Prion diseases encompass a heterogeneous group of fatal brain disorders associated with the accumulation of misfolded isoforms of the prion protein...

    Ryan R. Myers, Pedro Fernandez-Funez in Prions and Diseases

    Chapter 2023
    

17. Decoding the Cellular Trafficking of Prion-like Proteins in Neurodegenerative Diseases

    The accumulation and spread of prion-like proteins is a key feature of neurodegenerative diseases (NDs) such as Alzheimer’s disease, Parkinson's...

    Chenjun Hu, Yiqun Yan, ... Zhen Zhong in Neuroscience Bulletin

    Article 27 September 2023
    

18. Stem Cell Models in Prion Research

    Stem cells have the capacity to differentiate into the mature cells of any organ within the body. For this reason, they offer an interesting...

    Cathryn L. Haigh in Prions and Diseases

    Chapter 2023
    

19. Prion and Cancers

    Prion protein (PrP) is a glycosylphosphatidylinositol (GPI)-anchored, highly conserved, and ubiquitously expressed glycoprotein. In human, the PrP is...

    Wei **n in Prions and Diseases

    Chapter 2023
    

20. Molecular signatures in prion disease: altered death receptor pathways in a mouse model

    Background

    Prion diseases are transmissible and fatal neurodegenerative diseases characterized by accumulation of misfolded prion protein isoform (PrP

    Ranjit Kumar Giri in Journal of Translational Medicine

    Article Open access 27 May 2024