Human and experimental spongiform encephalopathies: recent progress in pathogenesis
Abstract
The spongiform encephalopathies belong to the group of "slow virus infections" of the nervous system, characterized by a long incubation period, a protracted course and involvement of the nervous system with a lethal outcome. In contrast to the conventional virus infections, such as visna in sheep and progressive multifocal leukoencephalopathy (PML) in humans, the etiological agent for the spongiform encephalopathies has not been clearly defined. The known forms in animals are scrapie in sheep and goats, transmissible mink encephalopathy, and chronic wasting disease of mule deer and elk. In humans, the three known forms are Kuru, now mainly of historical interest, Creutzfeldt-Jakob (CJ) disease and the syndrome of Gerstmann-Straussler-Scheinker (GSS). An important feature of these diseases is the lack of an immune response by the host, which is reflected in the absence of inflammatory infiltrates in the affected tissues. In this editorial the two most important hypotheses on the etiology and pathogenesis of this group of conditions will be discussed. The "prion" hypothesis considers the possibility that a protein, derived from a normal component of the neuronal membranes may have a leading role, not only in the infectivity and transmissibility of these diseases, but in the pathological changes that ensue. A single host gene would code for both the normal and altered proteins. The altered protein would be partially insoluble and would result in the deposition of fibrils and…
Read the paper
Related studies
- Regulation of nucleolus assembly by non-coding RNA polymerase II transcripts
- For a Healthy Histone Code, a Little SUMO in the Tail Keeps the Acetyl Away
- Discovering a binary CTCF code with a little help from BORIS
- Article Commentary: Stabilizing the Code–-Methods to Preserve RNA Prove Their Worth
- Foreword to the Special Issue for the RINTC (The Implicit Seismic Risk of Code-Conforming Structures) Project
- Commentary: Promoting Health and Well-being in Pediatric Primary Care Settings: Using Health and Behavior Codes at Routine Well-child Visits
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.