Progress on low susceptibility mechanisms of transmissible spongiform encephalopathies

Li-Li Qing1, Hui Zhao2, Lin-Lin Liu1

  • 1Laboratory of Conservation and Utilization of Bio-resources, Yunnan University, Kunming 650091, China.

Insights

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal neurodegenerative conditions. This review examines why dogs, rabbits, horses, and buffaloes show low susceptibility to these prion diseases.

Area of Science:

  • Neuroscience
  • Veterinary Medicine
  • Biochemistry

Background:

  • Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal neurodegenerative disorders affecting various mammals.
  • The "protein-only" hypothesis posits that prions, infectious particles lacking nucleic acid, cause disease through the conversion of cellular prion protein (PrP(C)) to a pathogenic form (PrP(Sc)).
  • Animal susceptibility to TSEs varies, with some species exhibiting low susceptibility.

Purpose of the Study:

  • To review and summarize recent research on the low susceptibility of specific mammalian species to TSEs.
  • To investigate the underlying protein structure, biochemical, and genetic factors contributing to this reduced susceptibility.
  • Focus on dogs, rabbits, horses, and buffaloes as models for low TSE susceptibility.

Main Methods:

  • Literature review of recent scientific studies on TSEs and prion disease.
  • Analysis of research focusing on the prion protein (PrP) structure and function in low-susceptibility species.
  • Examination of biochemical and genetic data related to prion protein conversion and disease resistance.

Main Results:

  • Identified key differences in prion protein (PrP) structure and biochemical properties among low-susceptibility species.
  • Highlighted specific genetic variations that may confer resistance to prion disease.
  • Provided a comparative overview of TSE susceptibility across dogs, rabbits, horses, and buffaloes.

Conclusions:

  • The low susceptibility of dogs, rabbits, horses, and buffaloes to TSEs is likely multifactorial, involving specific PrP structural and genetic characteristics.
  • Understanding these resistance mechanisms can inform future strategies for preventing and managing prion diseases.
  • Further research into the molecular basis of prion resistance is warranted.

Related Concept Videos

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...
898
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...
11
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining,...
10.1K
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...
7
Viral Meningitis01:18

Viral Meningitis

Viral meningitis is the most common form of meningitis and is often referred to as aseptic meningitis to indicate the absence of bacterial involvement. It is generally milder than bacterial meningitis, with symptoms including fever, headache, stiff neck, drowsiness, nausea, photophobia, and vomiting. Rarely, more severe manifestations or death may occur. Common causative agents include enteroviruses, particularly coxsackie A and B viruses and echoviruses, all members of the Enterovirus genus...
179
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...
52