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, M. Széll, L. Siklós, J. Engelhardt

Research output: Article

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 - máj. 2 2019

Fingerprint

Motor Neuron Disease
Amyotrophic Lateral Sclerosis
Motor Neurons
Intraperitoneal Injections
neurons
serums
Neurons
mice
injection
Immunoglobulin G
Serum
degeneration
Extremities
limbs
Calcium
calcium
Gliosis
Muscle Strength
Neuroglia
nerves

ASJC Scopus subject areas

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

Cite this

Experimental motor neuron disease induced in mice with long-term repeated intraperitoneal injections of serum from ALS patients. / Obál, Izabella; Nógrádi, Bernát; Meszlényi, Valéria; Patai, Roland; Ricken, Gerda; Kovacs, Gabor G.; Tripolszki, Kornélia; Széll, M.; Siklós, L.; Engelhardt, J.

In: International journal of molecular sciences, Vol. 20, No. 10, 2573, 02.05.2019.

Research output: Article

Obál, Izabella ; Nógrádi, Bernát ; Meszlényi, Valéria ; Patai, Roland ; Ricken, Gerda ; Kovacs, Gabor G. ; Tripolszki, Kornélia ; Széll, M. ; Siklós, L. ; Engelhardt, J. / Experimental motor neuron disease induced in mice with long-term repeated intraperitoneal injections of serum from ALS patients. In: International journal of molecular sciences. 2019 ; Vol. 20, No. 10.
@article{a7b390df58dc4369a78ca6d6d6af8dca,
title = "Experimental motor neuron disease induced in mice with long-term repeated intraperitoneal injections of serum from ALS patients",
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.",
keywords = "ALS, Degeneration, IgG uptake, Intracellular calcium, Motor neuron, Passive transfer, Serum",
author = "Izabella Ob{\'a}l and Bern{\'a}t N{\'o}gr{\'a}di and Val{\'e}ria Meszl{\'e}nyi and Roland Patai and Gerda Ricken and Kovacs, {Gabor G.} and Korn{\'e}lia Tripolszki and M. Sz{\'e}ll and L. Sikl{\'o}s and J. Engelhardt",
year = "2019",
month = "5",
day = "2",
doi = "10.3390/ijms20102573",
language = "English",
volume = "20",
journal = "International Journal of Molecular Sciences",
issn = "1661-6596",
publisher = "Multidisciplinary Digital Publishing Institute (MDPI)",
number = "10",

}

TY - JOUR

T1 - Experimental motor neuron disease induced in mice with long-term repeated intraperitoneal injections of serum from ALS patients

AU - Obál, Izabella

AU - Nógrádi, Bernát

AU - Meszlényi, Valéria

AU - Patai, Roland

AU - Ricken, Gerda

AU - Kovacs, Gabor G.

AU - Tripolszki, Kornélia

AU - Széll, M.

AU - Siklós, L.

AU - Engelhardt, J.

PY - 2019/5/2

Y1 - 2019/5/2

N2 - 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.

AB - 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.

KW - ALS

KW - Degeneration

KW - IgG uptake

KW - Intracellular calcium

KW - Motor neuron

KW - Passive transfer

KW - Serum

UR - http://www.scopus.com/inward/record.url?scp=85066944738&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85066944738&partnerID=8YFLogxK

U2 - 10.3390/ijms20102573

DO - 10.3390/ijms20102573

M3 - Article

C2 - 31130623

AN - SCOPUS:85066944738

VL - 20

JO - International Journal of Molecular Sciences

JF - International Journal of Molecular Sciences

SN - 1661-6596

IS - 10

M1 - 2573

ER -