Related Experiment Video

Updated: Aug 15, 2026

Determining Immune System Suppression versus CNS Protection for Pharmacological Interventions in Autoimmune Demyelination
09:38

Determining Immune System Suppression versus CNS Protection for Pharmacological Interventions in Autoimmune Demyelination

Published on: September 12, 2016

A role for dual viral hits in causation of subacute sclerosing panencephalitis

Michael B A Oldstone1, Samuel Dales, Antoinette Tishon

  • 1Department of Molecular and Integrative Neurosciences, The Scripps Research Institute, La Jolla, CA 92037, USA. mbaobo@scripps.edu

The Journal of Experimental Medicine
|November 2, 2005
PubMed

Insights

Researchers developed a new mouse model for subacute sclerosing panencephalitis (SSPE), a fatal brain disease caused by measles virus (MV). This model replicates key SSPE features, aiding future research into its causes and treatments.

Area of Science:

  • Neuroscience
  • Virology
  • Immunology

Background:

  • Subacute sclerosing panencephalitis (SSPE) is a fatal neurodegenerative disease linked to persistent measles virus (MV) infection in the central nervous system (CNS).
  • SSPE pathogenesis involves specific viral genome mutations, immune cell infiltration into the CNS, and high MV antibody titers.
  • Current understanding of SSPE's precipitating events and underlying biology is limited, with no effective treatments available.

Purpose of the Study:

  • To create a small animal model that accurately mimics the key pathological features of SSPE.
  • To provide a platform for investigating the molecular mechanisms driving SSPE pathogenesis.
  • To enable the testing of potential therapeutic strategies for SSPE.

Main Methods:

  • Generation of a transgenic mouse model expressing the measles virus (MV) receptor.
  • Transient immunosuppression of mice using lymphocytic choriomeningitis virus (LCMV) Cl 13.
  • Subsequent infection with MV to establish persistent neuronal infection.

Main Results:

  • The developed mouse model exhibited cardinal features of SSPE, including persistent MV infection in neurons.
  • Analysis revealed biased U-to-C hypermutations in the MV M gene within infected mouse brains.
  • Evidence of T and B lymphocyte infiltration into the CNS and high MV antibody titers in sera were observed.

Conclusions:

  • A novel transgenic mouse model successfully replicates the essential characteristics of subacute sclerosing panencephalitis (SSPE).
  • This model serves as a valuable tool for dissecting the molecular pathogenesis of SSPE.
  • The availability of this SSPE model facilitates the preclinical evaluation of novel therapeutic interventions.

More Related Videos

Intracerebroventricular and Intravascular Injection of Viral Particles and Fluorescent Microbeads into the Neonatal Brain05:51

Intracerebroventricular and Intravascular Injection of Viral Particles and Fluorescent Microbeads into the Neonatal Brain

Published on: July 24, 2016

Mouse Footpad Inoculation Model to Study Viral-Induced Neuroinflammatory Responses09:07

Mouse Footpad Inoculation Model to Study Viral-Induced Neuroinflammatory Responses

Published on: June 14, 2020

Related Concept Videos

Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
Arboviral Encephalitis01:25

Arboviral Encephalitis

Arboviral encephalitis refers to brain inflammation caused by arthropod-borne viruses, particularly those transmitted through mosquito vectors. Among these, West Nile virus (WNV), a member of the Flaviviridae family, is a significant public health concern. WNV is an enveloped, positive-sense, single-stranded RNA virus. Human infection typically begins when an infected mosquito introduces the virus into the dermis during feeding. The primary transmission cycle involves birds as amplifying hosts...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Subviral Agents01:29

Subviral Agents

Subviral agents are infectious entities that resemble viruses but lack one or more viral components, such as a capsid or essential replication machinery. These agents include viroids, prions, and satellites, each possessing distinct structural and functional characteristics that influence their mode of infection and replication.Viroids are the simplest subviral agents, consisting of circular, single-stranded RNA molecules without a protein coat. They exclusively infect plants, relying entirely...
Immune Response Against Viral Pathogens01:29

Immune Response Against Viral Pathogens

The immune system's response to viral infections is a complex and coordinated process involving natural killer (NK) cells, T cell-mediated responses, and antibody-mediated responses.
NK Cells
NK cells are a crucial part of our innate immune system, acting as the first line of defense against viral infections. These cells can recognize and kill infected cells without prior exposure to the virus, effectively slowing down the spread of infection. Additionally, NK cells produce proinflammatory...
Bacterial Meningitis II: Pathophysiology01:26

Bacterial Meningitis II: Pathophysiology

Bacterial meningitis typically begins when pathogens such as Neisseria meningitidis and Streptococcus pneumoniae colonize the nasopharynx and invade the bloodstream. This process is facilitated by bacterial virulence factors, such as polysaccharide capsules, which resist phagocytosis and complement-mediated killing. Less commonly, bacteria reach the central nervous system via contiguous spread from infections like otitis media or sinusitis, through congenital or acquired dural defects, or...