Experimental motor neuron disease induced in mice with long-term repeated intraperitoneal injections of serum from ALS patients

Izabella Obál, Bernát Nógrádi, Valéria Meszlényi, Roland Patai, Gerda Ricken, Gabor G. Kovacs, Kornélia Tripolszki, Márta Széll, László Siklós, József I. Engelhardt

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

In an earlier study, signs of commencing degeneration of spinal motor neurons were induced in mice with short-term intraperitoneal injections of immunoglobulin G (IgG) taken from patients with amyotrophic lateral sclerosis (ALS). Since in that study, neither weakness nor loss of motor neurons was noted, to test whether the ALS IgG in this paradigm has the potential to evoke relentless degeneration of motor neurons, treatment with repeated injections over a longer period was carried out. Mice were systematically injected intraperitoneally with serum taken from ALS patients over a 75-day period. At selected time points, the isometric force of the limbs, number of spinal motor neurons and their intracellular calcium levels were determined. Furthermore, markers of glial activation and the motoneuronal uptake of human IgG were monitored. During this period, gliosis and progressive motoneuronal degeneration developed, which led to gradual loss of spinal motor neurons, more than 40% at day 21, along with decreasing muscle strength in the limbs. The inclusion-like accumulation of IgG appeared in the perikarya with the increase of intracellular calcium in the cell bodies and motor nerve terminals. Our results demonstrate that ALS serum can transfer motor neuron disease to mice.

Original languageEnglish
Article number2573
JournalInternational journal of molecular sciences
Volume20
Issue number10
DOIs
Publication statusPublished - May 2 2019

Keywords

  • ALS
  • Degeneration
  • IgG uptake
  • Intracellular calcium
  • Motor neuron
  • Passive transfer
  • Serum

ASJC Scopus subject areas

  • Catalysis
  • Molecular Biology
  • Spectroscopy
  • Computer Science Applications
  • Physical and Theoretical Chemistry
  • Organic Chemistry
  • Inorganic Chemistry

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